COLUMBIA LIBRARIES OFFSITE HEALTH SCIENCES STANDARD HX00036293 PROPERTY OF Columbia University Eo. Crocker Special Research Fund €oUtqt of 3^l)fiitian& anb ^ux^toni ILihvavv TUMOURS INNOCENT AND MALIGNANT Digitized by the Internet Archive in 2010 with funding from Open Knowledge Commons http://www.archive.org/details/tumoursinnocentm1911blan TUMOURS INNOCENT AND MALIGNANT THEIR CLINICAL CHARACTERS AND APPROPRIATE TREATMENT BY Sir John BLAND-SUTTON, F.R.G.S. Surgeon to, and Member of the Cancer Investigation Committee of the Middlesex Hospital, etc. WITH THREE HUNDRED AND SIXTY ILLUSTRATIONS FIFTH EDITION NEW YORK FUNK AND WAGNALLS COMPANY PREFACE TO THE FIFTH EDITION Twenty years ago our knowledge of malignant tumours was unsatisfactory, especially in relation to tlie Cancer group. The description of cancer (or carcinoma) was founded on a study of this disease as it appears in the breast. It was not possible to write a satisfactory account of malignant disease occurring in organs like the kidney, uterus, thyroid gland, ovary or testicle. The clinical features and the modes of death not only vary according to the organ attacked, but are modified by the environment of the can- cerous organ. The enterprises of suigeons were limited until accurate information was forthcoming in regard to these matters, for a knowledge of the pathology of malignant growths is of prime importance to those engaged in the operative treatment of these diseases. Since the first appearance of this book (1893) a large amount of investigation has been carried out in relation to cancer of individual organs. The frequency with which it arises in the gall-bladder has come as a surprise to physi- cians as well as to surgeons. Another new thing is the discovery that the Fallopian tube is liable to be the seat of primary cancer. Among more recent additions to our knowledge of cancer is the recognition of the fact that cancer of the ovary is due to implantation of cancerous particles shed from a primary focus in the breast, the colon, the stomach, or the gall-bladder, or poured out from the open mouth of a cancerous Fallopian tube. Informa- tion on these things, as well as much new matter bearing on the cancer problem, is incorporated in this edition. 47, Brook Street, Grosvenor Square, W. January 1911. EXTRACT FROM THE PREFACE TO THE FIRST EDITION In 1885 I began to collect materials, from man and other vertebrates, in order to make myself acquainted with the histological peculiarities of tumours. Attention was first devoted to Cysts, and the results of the investigation were embodied in lectures delivered at the Royal College of Surgeons during the years 1886-91 : they dealt particularly with the group of tumours known as Dermoids and Tubulo-Cysts. During the same period I contributed to the Odontological Society of Great Britain a series of papers to show that many tumours of the jaws, classed as exostoses, are derived from aberrations of teeth. Whenever it seemed desirable to illustrate the nature of a genus of tumours by reference to Comparative Pathology, I have not hesitated to do so. Without this aid, any attempt to catch the deeper meaning of many tumours is as difficult as endeavours to decipher a palimpsest in which the first characters, written in an unknown tongue, have been im- perfectly removed from the parchment, and are allowed to mingle with the second inscription. CONTENTS Introduction . . . . 1 CHAPTER 1. Lipomas . 12 2. Chondromas . 26 3. Osteomas . 34 4. Myelomas . 45 5. Sarcomas . 52 6. Sarcomas (continued) . 62 7. Sarcomas of Bones . 77 8. Sarcomas of Glandular Organs . 95 9. Tumours of the Adrenal . 106 10. Pigmented Tumours . 110 11. Moles ..... . 123 12. Neuromas .... . 130 13. Neuromas (continued) . . 140 14. Neuromas (concluded) : Gliomas . 148 15. Angeiomas and Lymphangeiomas . 156 16. Uterine Fibroids . . . . . 168 17. Uterine Fibroids (continued) . 180 18. Uterine Fibroids (continued) . 187 19. Uterine Fibroids (continued) . 195 20. Uterine Fibroids (concluded) . 206 21. Odontomas . 211 22. Papillomas .... . 234 CONTENTS CHAPTER 23. Horns 24. Adenoma . • 25. Carcinoma 26. Concerning the Cause of Cancer 27. Treatment of Malignant Tumours 28. Carcinoma of the Breast . 29. Carcinoma of the Breast {concluded) 30. Epithelial Tumours of Sebaceous Glands 31. Epithelial Tumours of the Thyroid Gland 32. Carcinoma of the Lips, Mouth, Tongue, etc. 33. Carcinoma of the O^Isophagus, etc. 34. Epithelial Tumours of the Liver, etc. 35. Carcinoma of the Urinary Organs, etc 36. Epithelial Tumours of the Uterus . 37. Epithelial Tumours of the Uterus (conti 38. Uterine Fibroids and Cancer 39. Papilloma and Carcinoma of the Tube 40. Endotheliomas 41. Chorion-Epithelioma .... 42. Teratomas 43. Teratomas [concluded) 44. Sequestration Dermoids 45. Sequestration Dermoids (concluded) . 46. Tubulo-Dermoids 47. Cervical Fistul^e, etc 48. Tumours of the Ovary 49. Tumours of the Ovary {continued) . 50. Tumours of the Ovary {continued) . 51. TuMOLTis OF THE OvARY {continued) . nued) 242 248 254 275 287 292 303 307 313 319 328 345 360 372 380 393 398 405 415 422 433 442 455 466 474 486 502 509 520 CONTENTS CHAPTER 52. Tumours of the Ovary {concluded) 53. Tumours of the Testicle 54. Heterotopic Teeth 55. Retention-Cysts 56. Retention-Cysts {concluded) 57. Tueulo-Cysts . 58. Hydrocele 59. Pseudo-Cysts . 60. Pseudo-Cysts {continued) 61. PsEUDO- Cysts {continued) : Neural Cysts 62. Pseudo-Cysts : Neural Cysts {concluded) 63. EcHiNOCoccus Disease .... Index XI PAGE 529 537 553 563 575 594 602 611 620 629 638 652 675 TUMOURS: INNOCENT AND MALIGNANT INTRODUCTION It has long been customary in surgical writings to group together a ve^ry heterogeneous assembly of morbid con- ditions under the term Tumours. This is a very ancient name, and merely means a swelling, but the careful micro- scopic investigations of morbid anatomists with the aid of differential staining (histologic chemistry), and the study of the relationship of micro-organisms to many swellings called tumours, have led to a revolution in our knowledge, so that the term has been stripped of its former wide significance. In clinical work, the word tumour is not likely to disappear, although it has lost its importance to the pathologist. Formerly, the term tumour was applied to the abnor- mal swellings which characterize the gummatous stage of syphilis ; the lesions of actinomycosis, leprosy, and other diseases, collectively known as the Infective Granulomas ; the excessive formation of callus around the fragments of broken bones, and the exuberant production of cicatricial tissue known as keloid. It is noteworthy that almost every increase in our knowledge regarding the cause of tumour-diseases results in reducing the list of morbid con- ditions known as tumours, either by removing some from this category, or by combining under one term a number of apparently diverse conditions which were formerly regarded as independent. Tumour-diseases of the nervous system illustrate this. Among recent evictions from tumours is the big prostate of advanced life, which is usually classed among adenomas ; but some excellent B 2 ' INTHOBTJGTION observations indicate that this disease, the bane of elders, is due to micro-organisms which gain access to the glandular recesses of the prostate from the urethra and set up inflam matory (reactive) changes. In ignorance of the cause of tumours (pathogenesis), we fall back on their minute structure (histology) as a basis of classification (taxonomy). This is the natural outcome of the careful investigation of the minute structure of tumours, because it led investigators to realize that they consisted of the same tissues which compose the normal organs of the body. This was a great step. Anatomic ob- servations taught men that animal bodies are made up of diverse structures, such as fat, suet, bone, gristle, muscle, tendon and the like, but the microscope revealed that they are composed of fundamental tissues, which enter into the construction of organs of the most diverse form and func- tion. The base is the connective tissues, comprising bone, fat, cartilage, etc., and two remarkable structures known as muscle and nerve. There is also a peculiar material which permeates the body and enters into the composition of every organ; it is called areolar tissue, a ubiquitous web which is stout and strong as fascia and periosteum, ex- tremely delicate in the nervous system, and so fine in the retina as to need careful preparation to render it perceptible to the microscope. The connective tissues form the frame- work of the body, and constitute a sort of sustentaculum in compound organs, such as the liver, intestines, kidney, and the like, for the support of epithelium, and serve as a naesh in which blood-vessels and lymphatics can ramify to supply the liquid tissue — blood, from which the epithelial cell can obtain material to form the secretion which it is the function of particular glands to elaborate. The careful and critical study of the minute (micro- scopic) structure of tumours having revealed that they were composed of tissues normally existing in the animal body, pathologists realized that the histology and embryology of an organ enable an experienced oncologist to predict the various genera of tumours and cysts to which it may be liable. Thus the tibia of a child contains cartilage, bone, fibrous tissue, young connective tissue, fat, and red marrow. LIABILITY OF ORGANS 3 The epiphysial cartilages are the source of chondromas ; the bone furnishes osteomas, the periosteum sarcomas, and very rarely lipomas, and myelomas arise in the red marrow. Cancers do not arise primarily in bone, as it lacks epithelium, but they often occur in bone as secondary deposits. Although our knowledge of the intimate structure ot tumours, thanks to differential staining methods, is now sutficient to enable us to indicate from the structure of an organ the genera of tumours to which it may be liable, nevertheless, the most careful study of the minute struc- ture of such organs as the salivary glands would not lead us to suspect their liability to pure chondromas ; and it is strange that" they should occur in the parotid, sub- maxillary, and lachrymal glands, and yet be unknown in the pancreas. What oncologist, merely from studying the histology of a normal ovary, would suspect that it would be the point of origin of a dermoid ? It is like studying the fauna of a country. For instance, who imagined, until Australia was discovered, the existence of extraordinary mammals like kangaroos and duck moles ? But knowledge gained from observation enables us to state that gliomas do not arise in bone, nor myomas in the brain, nor der- moids in the spleen, liver, or kidney, with the same cer- tainty that we assert that at the present period of our planet's history lions do not sport about the ice-fields of Greenland, nor do humming-birds flit about the flower-beds of Hyde Park. It is, however, necessary to point out that, although the tissues of an organ determine the genera of tumours to which it may be liable, their relative frequency can onlv be gathered from observation. The variations in the liability of the organs of the body to tumours are a very curious matter. The heart is very rarely occupied by a tumour ; on the other hand, the uterus, also a muscular organ, is with extreme frequency the seat of fibroids. The liability of bones to sarcomas is proverbial, yet a sarcoma of a voluntary muscle is most uncommon. A primary tumour is a rarity in the lung, but it is common enough in the brain or the eyeball. 4 INTBOBUGTlOK Sarcomas are frequent in the kidneys, but a primary sar- coma in the Hver or spleen is extremely rare. These and many kindred questions indicate profound imperfections in our knowledge concerning the cause of tumours. It may be stated, without fear of contradiction, that no one has succeeded in framing a satisfactory classification of tumours. In this Avork the subjoined plan will be followed : — Group I. Tumour-diseases of the connective tissues. This will include Lipomas, Chondromas, Osteomas, Myelomas, Sarcomas, Myxomas, Myomas, Neuromas, Angeiomas, Endo- theliomas, and Uterine Fibroids. Group II. Tumour-diseases of teeth. Odontomas. Group III. Epithelial tumours. This comprises Papillomas (warts), Adeno- mas, Carcinomas. Group IV. Endotheliomas. This includes Hsemendotheliomas, Lymphen- dotheliomas. Peritheliomas (Angeio- sarcomas). Group V. Tumours arising from the fcetal membranes. Chorion-epitheliomas (Deciduomas). Group VI. Teratomas. Dermoids. Embryomas. Group VII. Cysts. Tumours have from very early times been arranged into a mahgnant and an innocent or benign division, based on the knowledge gained from observation that some of them inevitably destroy life, w^hilst others do not disj^lay such destructive propensities. It is important to remember that benign tumours may, and often do, destroy life. The essential difference between an innocent and a malignant tumour may be expressed thus: The baneful effects of innocent tumours depend entirely on their environment, but malignant tumours destroy life whatever their situation. Environment in relation to tumours. — Although throughout the whole of this book reference will be made ENVIRONMENT 5 to the destructive effects of turaonrs of all kinds, which will make the reader realize the truth of the words that Byron puts into the mouth of Werner, " Death hath a thousand gates," it will, perhaps, be useful to describe some examples which illustrate the importance of environ- ment. A small tumour occupying a vital organ will sometimes destroy life from mechanical causes. For example : A girl aged 14 was seized with paraplegia and died in ten days. The cervical segment of the cord contained a tumour of the size and shape of a small olive (Fig. 1). Some of the most tragic deaths due to tumours struc- turally benign occur in connexion with the air-passages. A man aged 76 entered a restaurant and made an incoherent noise and motion of his hand, which was taken to be a request for water. Death took place quite sud- White matter denly. At the post-mortem ^'^Tumour- examination an ovoid tumour, IJ inches in its greatest diame- ter, was found growing from the left gloSSO-epiglottic folds. Fig. l.— Cervical segment of the cord The larynx and tongue with in transverse section showing a ■^ , . o tum^our m the grey substance. the tumour in situ were sent to the museum of the Royal College of Surgeons. Shattock reported the tumour to be a lipoma, and suggests that, from some unusual act on the part of the patient, it became engaged in the grasp of the pharyngeal constrictors and suffocated him as he involuntarily attempted to swallow his own tumour. A man 36 years of age was found lying on his back in a street adjacent to the Middlesex Hospital, apparently in a fit; when brought into the casualty-room he was dead. At the post-mortem examination a tumour was found connected with the cervical section of the windpipe, embedded in a thick fibrous capsule, its inner segment being firmly fixed to the trachea between the fourth and ninth semi-rings. The tumour, including its capsule, was somewhat larger than a dove's q^^ (Fig. 2), and it had severely compressed the trachea (Fig. 3). The tumour presented the microscopic structure peculiar 6 INTEOBUGTION to the parathjrroid body. It was probably an enlarged para- thyroid. Little was known of these peculiar bodies when this specimen came to hand in 1886. The capsule consisted of dense laminse of fibrous tissue ; the tumour could have been easily enucleated from its capsule. The preceding examples show that the tumours classed as benign or innocent are only dangerous when from their Fig. 2. — An enlarged and encapsuled parathyroid body. It com- pressed the trachea and produced fatal dyspncea. position they mechanically interfere with vital organs, or obstruct functions necessary to the maintenance of life. Malignant tumours, on the other hand, destroy life in whatever situation they arise. Melanomas illustrate this very well. A man 50 years of age came under my ob- servation with an intra-ocular tumour no larger than a cherry-stone growing from the uveal tract. The eyeball was promptly excised, and the tumour, which in this case had a deep black hue, had remained strictly confined to the globe. Within two years this man died with secondary ENVIRONMENT 7 tumours in the liver and many other organs ; his skin turned quite black, melanin appeared daily in the urine, and the free fluid in his belly also contained pigment in abundance. Although it is true that malignant tumours destroy life in whatever situation they arise, nevertheless environ- ment exercises great influence on the rapidity as well as on the mode in which they kill. For instance, a cancer of the larynx may cause death from suffocation, but it is more frequently fatal by setting up septic pneumonia in consequence of the inhalation of septic matter from the sloughing surface of the growth. Cancer of the gastric Fig. 3. —Section of an enlarged parathyroid body and trachea, showing the amount of stenosis. (iV«^. size.) orifices usually entails death from starvation, and malig- nant disease of the prostate destroys life by leading to renal disorders consequent upon impediment to the free escape of urine from the bladder. It may be stated almost as an axiom that when a malignant tumour implicates a vital organ it will often destroy life before there has been time for dissemination. When the environment has been unfavourable in this respect death is usually induced by secondary nodules occupying important organs, e.g. lungs, liver, brain, etc. This is a matter which will receive careful attention in the description of malignant disease as it attacks different organs. It is, however, a very remarkable fact that a peri- osteal sarcoma of the femur is the most deadly tumour which attacks the human frame, but a sarcoma of the 8 INTRODUCTION tibia with the same histologic characters will, with pre- cisely the same treatment (amputation), take as many years to destroy life as the tumour of the femur requires months. This would appear to indicate that the two tumours, though structurally alike, really have different causes, yet there are facts which lead us to suspect that variations in tissue actually constitute an altered environment. The only condition which supports this view in a positive way is echinococcus-disease. The final chapter of this book con- tains abundant evidence as to the effects of environment on the character of echinococcus colonies, besides illustrat- ing the varied manner in which the surroundings deter- mine the mode by which these parasites often induce the death of human beings, their involuntary intermediate hosts. Some of the most terrible examples illustrating danger- ous environment are inconspicuous solid ovarian tumours and dermoids, incarcerated in the pelvis by a gravid uterus. In many instances the presence of a tumour is unsus- pected even when the woman has been hours in labour. Obstruction of this kind is very fatal to the child and often to the mother, and the injuries Avhich women sus- tain in such circumstances are often of an appalling character, as works on midwifery testify. Even when ovarian tumours do not obstruct delivery, their co-exist- ence with pregnancy is an inimical condition, and may bring about the death of the mother either in the progress of the pregnancy, during labour, or in puerpery. There are some anatomical conditions which distinguish innocent from malignant tumours : those that are benign usually possess an investing membrane, or capsule, by which they are isolated from the tissues in which they grow; they do not infect lymph-glands, nor recur after complete removal, and rarely imperil life save when grow- ing in connexion with, or in the immediate vicinity of, vital organs. Malignant tumours, on the other hand, are rarely encapsuled, and tend to infiltrate the surrounding tissues ; they infect the lymph-glands which receive the lymphatics from the part affected, are exceedingly liable to recur after removal, tend to become disseminated by the lymph- and blood-stream, and inevitably destroy life. AGE-DISTRIBUTION 9 Age-distribution. — Although some tumours may occur at any period of life — e.g. fatty tumours and sarcomas — the majority of the genera have a fairly well-defined, and occasionally a very strict age-limit. For example, the species of tumour known as glioma, which arises in the retina, has rarely been observed after the twelfth year ; it is peculiarly limited to infants, and this is also the case with the remarkable condition known as "gliomatous disease" of the pons and medulla. Myelomas are tumours of adolescence ; and this is true of odontomes, for they only arise in connexion with the germs of the permanent teeth. Uterine fibroids are produced during menstrual life, and careful inquiry demonstrates that the dread chorion-epithelioma (deciduoma) is a by-product of concep- tion, and therefore restricted to the child-bearing period of life. Parovarian cysts do not occur before the fifteenth year, and papillomatous cysts of the ovary are fairly well distributed to the three decades bounded by the twenty- fifth and fifty-fifth years. Angeiomas and sequestration dermoids are essentially congenital tumours, whilst melano- mas are almost confined to adults. It may with truth be stated that age constitutes an environing condition when we reflect that sarcoma in in- fancy tends to be bilateral — e.g. when it attacks the kidneys, eyes, adrenals, or ovaries. In adult life sarcoma of these same organs is invariably unilateral; but, apart from this peculiarity, as many of the subsequent chapters will show, the tumours at these diverse periods of life exhibit obvi- ous and unmistakable differences in their minute structure. Multiplicity. — Innocent tumours are often multiple : five, ten, or twenty lipomas on an individual are not un- common numbers. A thousand neuromas have been counted on one patient ; a hundred fibroids may grow concurrently in the tissues of the uterus, and ten adenomas occasion- ally occupy a single thyroid gland, but the occurrence of two primary cancers in the same patient is excessively rare, with the exception of the peculiar variety known as rodent cancer. The co-existence in the same person of two genera of mnocent tumours is well known — indeed, is almost a matter 10 INTRODUCTION of daily observation, uterine fibroids and ovarian dermoids, lijDomas and sequestration dermoids, chondromas and osteo- mas being frequent combinations. An individual may have one or more innocent tumours for many years, and then a carcinoma may arise, some- times in an organ abeady occupied by a tumour. For example, the uterus may be the seat of a large fibroid, and carcinoma may subsequently arise in the endometrium. Carcinoma and adenoma occasionally grow concurrently in the same breast ; or cancer may arise in the mamma a year or more after the removal of an ovarian tuinour. The transformation of innocent into malignant tu- mours. — A long study of the histogenesis of tumours has convinced the writer that the clearly innocent and the decidedly malignant tumours present distinct histologic features, but there are intermediate varieties which cannot be sharply defined in relation to these points, and this comes out in a striking and suggestive way when an in- dividual possesses tumours of a supposed innocent genus in multiples : for example, from uterine fibroids, when they are multiple, a tumour may be selected which sometimes re- quires a saw to divide it ; another may be as soft as a ripe fig, and a third will be as viscous as jelly and almost diffiuent : a soft fibroid of this character Avill sometimes recur after enucleation. Careful records are accessible in which fibroids of apparently simple structure have dissemi- nated and destroyed life ; it should be borne in mind that the uterus is liable to be the seat of a sarcoma which, in the early stages, mimics a fibroid in its naked-eye characters. It is so difficult to decide between the slow-growing spindle-cell sarcoma, the fibrifying sarcoma, and the gela- tinous fibroid (myxoma) that it is unwise to argue from our present knowledge that innocent connective-tissue tumours may undergo transformation into sarcomas until distinctive methods have been introduced by the histologist, chemist, biologist, or bacteriologist. It may be stated that every genus of the connective-tissue group, with the exception of the lipomas, presents varieties which shade away indefi- nitely from the typical species towards the sarcomas, and display malignancy. It is also clear, from a careful study of MALIGNANT TRANSFORMATION 11 the histology of tumours, that the more perfectly they ap- proach in type normal tissues the more benign is their clinical conduct; and the more widely the tissues of a tumour depart from the normal elements in which they arise, so much more likely are such tumours to be malignant. It may be stated that a wide departure from the normal type of tissue in a given tumour expresses the degree of malignancy. Certainly the more widely the cells of a tumour deviate from those normal to the matrix in which it grows the more rapidly do they multiply ; and this persistent cell- proliferation is one of the most obvious features of mahg- nancy. The more carefully the histology of tumours is investigated, the more obvious is it that the border- land between innocent and malignant species becomes less easily definable. This has been very definitely revealed in the case of ovarian dermoids ; few tumours had a better reputation for innocency, yet we now know that the less typical forms are liable to infect the peritoneum and even dis- seminate, and some varieties of testicular embryomas are among the most malign tumours that attack mankind. Kealizing the uncertainty attending the diagnosis and prog- nosis of tumours and tumour-diseases, pathology confirms the practice advocated by surgeons in dealing with them, namely, removal, whenever practicable, at the earliest possible moment. GROUP L CONNECTIVE-TISSUE TUMOURS CHAPTER I LIPOMAS (FATTY TUMOURS) A LIPOMA is a tumour composed of fat; the genus consists of a single species. With the exception of sarcoma it is the naost generalized genus of tumours which occurs in man. It therefore will be convenient to consider lipomas according to the situations in which they arise, such as the subcutaneous and subserous tissues ; beneath synovial or mucous membranes ; between or even in muscles; or in connexion with periosteum, and the meninges of the brain and spinal cord. The distribution of fat in the animal body is comparable to that of starch in the vegetable kingdom, where it also takes on a tumour-like form, as in conns and tubers (Shattock). 1. Subcutaneous lipomas. — Beneath the skin there exists a layer of fat which varies in thickness in different parts, but is most abundant over the trunk and trunk- ends of the limbs. This subcutaneous fat is a common situation in which to find fatty tumours. Usually they occur as irregularly lobulated encapsuled tumours, more or less adherent to the skin : unless they have been irritated, lipomas are movable within their capsules. Generally one lipoma is present, but two, ten, twenty, or more may occur concurrently^ on the same individual. In size they vary widely; a lipoma weighing sixteen ounces is a tumour of fair size ; exceptional specimens have been reported to weigh fifty, eighty, and even one hundred pounds. Although subcutaneous lipomas are for the most part confined to the trunk and trunk-ends of limbs, they may arise on the distal parts of the limbs, such as the hands and feet (Figs. 4 and 5). Many specimens have been 12 LIPOMAS 13 observed in the palm of the hand, a situation in which they are apt to give . rise to difficulty in diagnosis, more especially as they simulate compound ganglia of the flexor tendons. The lobes of fat are apt to burrow beneath the palmar fascia, and it is probable that some lipomas of the palm originate beneath this fascia, in the lobules of fat lying between the lumbricales. A lipoma has been observed on the back of the hand of a boy 8 years old. Fig. 4. — Lipoma of the sole which had existed for thirty years. It was removed by Percivall Pott. {Musetim of St. Bartholomeic'' s IlospitaL) and a process of the tumour passed between the third and fourth metacarpals into the palm (Pupovac). Fatty tumours are occasionally found on the fingers : Steinheil has col- lected a large number of examples. A lipoma in the sole is more comprehensible than one in the palm, yet, strange to relate, the}'" are far more frequent in the hand than in the foot ; in both situations they are apt to be congenital, and nearly always cause doubt in diagnosis (Gay, Lockwood). Fatty tumours are rarely met with upon the head or 14 GONNEGTIVB-TISSUE TUMOURS face, but I have on three occasions removed a lipoma from beneath the skin covering the temporal fascia. There is a variety of fatty tumour sometimes called, on account of its vascularity, ncevo-lipoma : this may be a nsevus which has undergone fatty degeneration. Probably some of the vascular lipomas which occasionally occur on the face are of this nature. Fatty tumours which have existed many years some- Fig. 5. — Lipoma in the palm. times calcify, the earthy matter being deposited in the fibrous septa of the tumours. A partially calcified lipoma is preserved in the museum of St. Bartholomew's Hospital, which came from the arm of an Arab sheikh, where it had existed fifty years. Calcification may be associated with saponification of the fat. The subcutaneous fat in the neck, axilla, and groin sometimes forms irregularly lobulated masses called diffuse lipomas, but they are not strictly tumours (Fig. 6). LIPOMAS 15 2. Subserous lipomas. — The peritoneum, like the skin, rests upon a bed of fat, the thickness of which varies con- siderably. Lipomas occurring in subserous tissue are sessile, or pedunculated. Surgeons have long been aware, in operating for in- guinal or femoral hernia, that occasionally they come across a mass of fat and find difficulty in determining whether Fig. 6. — Diffuse lipoma of the neck. [After Morrant Baker.) it be omental or a local increase of the subserous fat sur- rounding the hernial sac. It is now clear that in the neighbourhood of the femoral and inguinal canals an over- growth of the subserous fat may occur and be mistaken for a hernia, and individuals have been recommended to wear, and have actually worn, trusses for fatty masses of this character. It is also clear that, as these local over- growths of fat arise and protrude in the groin, they occasionally draw with them a pouch of peritoneum un- associated with a hernia. These pouches may afterwards 16 G0NNEGTIVE-TI8SUE TUMOURS lodge a piece of gut, and become true hernial sacs. Thus peritoneal pouches, produced mechanically by subserous lobes of fat, may subsequently become hernial sacs ; on the other hand, pedunculated lobes of fat may arise in relation with peritoneal pouches which were originally hernial sacs. In some cases a subserous lipoma of this character will invagi- nate a peritoneal pouch and form a pedunculated tumour within the hernial sac. More rarely a fatty tumour will arise in connexion with the spermatic cord. Gabryszewski has collected the more important cases, and discussed the difficulty such tumours cause in diagnosis. Andrewes found a tumour which appeared to be a lipoma of the sper- matic cord, but on microscopic examination it exhibited the structure of an adrenal. Fatty tumours arise in the scrotum or labium without being connected with hernial pouches (Hutchinson). Lipomas arising in the subperitoneal tissue occasionally appear in the anterior abdominal wall, especially near the umbilicus ; they are known as " fatty hernise of the linea alba," and are frequently associated with peritoneal pouches. Fatty tumours sometimes grow between the layers of the mesometrium, and in some instances are so large as to simulate ovarian tumours (Parona, Treves). Masses of fat, in many respects resembling the so-called " diffuse lipoma " of the subcutaneous tissue, have been re- moved from the abdomen, weighing thirty and even fifty pounds (Pick, Cooper Forster). Hernial lipomas are interesting, for they explain the mode in which appendices epiploicse arise : these are localized pedunculated overgrowths of subserous fat, and are particularly large and arborescent in the neighbourhood of an old syphilitic stricture of the rectum. In well-nourished individuals the fat of the appendices epiploicse is directly continuous with the fat in the layers of the mesentery; when wasting occurs the fat between the appendices and the mesentery is liable to atrophy and to leave an adipose nodule at the bottom of a peritoneal pouch. The movements of the intestine and the traction of the nodule lead to the formation of a pedicle which often becomes LIPOMAS 17 twisted ; sometimes tlie pedicle is so thin that it breaks, and the appendix is set free. Pieces of fat, not infrequently calcified, detached in this way, have been found in hernial sacs. A fatty tumour may arise in the fat behind the ensiform cartilage, and, extending through the gap in the diaphragm in this situation, occupy the lower end of the anterior mediastinum. Rokitansky pointed out that the subpleural fat in the intercostal region sometimes forms a lobulated mass which prolapses into the sac of the pleura. C. Gussenbauer has described and figured a subpleural lipoma which made its way on each side of the ribs. The two lobes were joined by a narrow isthmus so as to form an intra- and an extra- thoracic portion; the latter bulged under the pectoralis major and simulated a sarcoma. 3. Submucous lipomas. — Fat exists in submucous tissue in many situations, and, like that in the subcutaneous tissue, is not infrequently the source of lipomas. (a) Subconjunctival lipomas. — These occur near the line where the conjunctiva is reflected from the lower lid to the eyeball ; they are almost entirely confined to chil- dren. Fatty tumours sometimes arise from the orbital fat and cause the conjunctiva to protrude in the neighbour- hood of the lachrymal gland and near the insertions of the ocular muscles. (6) The lips. — Lipomas in this situation are very rare and never large (Edmunds). (c) Laryngeal lipomas. — A few remarkable examples have been reported. Holt met with a pedunculated lipoma 22-5 cm. in length, which grew from the side of the left aryteno-epiglottic fold and extended into the cesophagus. Sidney Jones removed a lipoma from the right aryteno- epiglottic fold of a man 40 years of age. The patient could protrude the tumour into his mouth. (See also Shattock's case, p. 5.) (d) Gastric lipomas. — Virchow has figured a lipoma which grew beneath the mucous membrane near the pylorus ; it was as big as a nut. (e) Intestinal lipomas. — A submucous fatty tumour of the small or the large intestine is very rare, and in both c 18 G0NNEGTIVE-TI8SUE TUMOURS situations may be occasionally inimical to life. The danger of a lipoma of the ileum is well set out in a case recorded by Stabb ; the tumour arose in the submucous tissue 75 cm. from the ileo-csecal valve ; in size and shape it resembled three acorns conjoined at the cups, and it caused intussus- ception of the bowel. The invagination was reduced and the tumour excised. Unfortunately the mucous membrane sloughed, and the patient, a man of 32 years, died. The specimen is preserved in the museum of St. Thomas's Hospital. I successfully removed from a man 44 years of age a lipoma, weighing two ounces, which occupied the submucous tissue of the ascending colon, 5 cm. above the ileo-csecal valve. The patient had passed through several acute attacks of intestinal obstruction. During the operation in this case I saw that the serous coat over the tumour was dimpled. Stabb noticed the same condition in his case, so that it is quite possible that these lipomas, though pro- jecting into the gut, really arose in the subserous stratum of fat. Submucous fatty tumours have been observed on several occasions in the jejunum and colon. The great danger is, of course, their liability to obstruct the intestine. The literature of intestinal lipomas has been collected by Hillier, Langemak, and Shattock. 4. Subsynovial lipomas. — Beneath the subserous tissue of large joints, such as the knee, there is a layer of fat of varying thickness. This fat may, as in the case of inguinal lipomas, increase in quantity and, projecting into the joint, form a fatty tumour. A common situation for this to occur is beside the patella, at the spot normally occu- pied by the alar ligaments. Many specimens are doubtless due to overgrowth of the fat in the alar fringes, but they may arise in other parts of the joint. The best-known variety of subsynovial fatty tumour is that to which Mtiller applied the term "lipoma arbor- escens." This condition is often, but by no means always, associated with rheumatoid arthritis. A typical specimen (Fig. 7) consists of small finger-like processes of fat pro- jecting into the cavity of the joint ; each process is covered LIPOMAS 19 with synovial membrane. The hpoma arborescens bears pre- cisely the same relation to the synovial membrane that the appendices epiploicse bear to the peritoneal investment of the colon and its sigmoid flexure. 5. Intermuscular lipomas. — Fatty tumours now and then arise in the connective tissue between muscles ; they have been found between the greater and lesser pectorals, Fig. 7. — Lipoma arborescens of the shoulder-joint. A, Acromion. C, CoracoiJ. F, Glenoid fossa. between the muscles of the tongue, and the intermuscular strata of the anterior abdominal wall. In the last-men- tioned situation they have been known to attain prodigious proportions. Exceptional examples have been described by Astley Cooper, Eve, and others. The most remarkable example of this variety of lipoma arises in connexion with the sucking-cushion (Fig. 8). This curious ball of fat is situated between the masseter and 20 00NNEGTIVE-TIS8UE TUMOUBS buccinator muscles, and comes into close relation with the buccal mucous membrane. It is believed to play an impor- tant function in connexion with sucking, by distributing atmospheric pressure and preventing the buccinators from being forced between the alveolar arches when a vacuum is created in the mouth. These cushions are relatively much larger in infants than in adults. Ranke also points out that in emaciated children they are only slightly diminished in size, even when there is scarcely any subcutaneous fat. f y/ Pig. 8. — Emaciated child crying and displaying sucking- cushions. {After Ranke.) The hibernating gland. — In animals which pass the winter in sleep, such as the hedgehog, dormouse, and marmot, masses of fat accumulate in the neck and under the scapula as winter approaches ; this fat dwindles during hibernation, and disappears at the advent of spring. Hatai and Shattock have come independently to the conclusion that the fat of this hibernating gland differs in some of its microscopic characters from common fat. In the human subject they find that some of the deeply seated fat in the neck corresponds in disposition and structure to that of the hibernating or interscapular gland. This layer of fat exists in the normal human foetus before term (Fig. 9). LIPOMAS 2J 6. Intramuscular lipomas. — Many examples of fatty tumours occurring in the midst of muscles have been reported, and are of interest from the trouble they cause in diagnosis. They have been found in the deltoid, biceps humeri, complexus, and rectus abdominis ; and in the middle of a submucous fibroid of the uterus (T. Smith, Lebert). Fig. 9. — A foetus of ij^ months dissected to show the hibernating gland. (After Bomiot.) The condition described as fatty tumour of the heart is simply overgrowth of the fat occupying the auriculo-ven- tricular grove. 7. Parosteal lipomas. — This term has been applied to fatty tumours arising from the periosteum of bone. When congenital, they nearly always contain tracts of striated nmscle fibre. Some of these tumours are clinical puzzles. Fatty tumours have been found growing from the peri- osteum of vertebrse, the femur, tibia, fibula, clavicle, scapula, radius, coccyx, ischium, spine of ilium, and body of the pubes. 22 CONNECTIVE-TISSUE TUMOURS 8. Lipomas on nerves. — Occasionally a fatty tumour arises from the sheath of a peripheral nerve ; this is a neuro-lipoma. There is a specimen in the museum of the Middlesex Hospital which grew from the sheath of the median nerve as it escaped from the anterior annular ligament into the palm. It was situated entirely beneath the palmar fascia. Vickery succeeded in removing a lipoma weighing 12| ounces from the thigh of an infant 9 months old. The tumour grew from the sheath of the great Fig. 10. — Infant 9 months old witli a large lipoma growing among the hamstring muscles. It was successfully removed. {After Vickery.) sciatic nerve. Before operation the growth simulated a sarcoma. (Fig. 10.) 9. Meningeal lipomas. — Fatty tumours occur within the spmal dura mater, as well as externally to this membrane. When growing within the sheath they surround the cord: Gowers, Recklinghausen, and Obre have recorded examples. In the cases described by the first two observers the tumours contained striped muscle tissue. The occurrence of an intra- dural lipoma is not surprising, as the loose connective tissue between the cord and dura mater contains fat. Fatty tumours are not uncommon in the middle line of the back, especially in the lumbo -sacral region, overlying the sac of a spina bifida. (Fig. 11.) LIPOMAS' 23 A lipoma has been observed encapsuled between the layers of the dura mater lining the sella turcica ; it extended into the middle fossa of the skull on the left side. The patient, who was a woman 44 years of age, suffered from periodical pain in the head, and eventually from ptosis (two years). The tumour was as big as a hen's egg. Fig. 11. — Meningeal lipoma overlying the sac of a spina bifida. {Museum, Royal College of Surgeons.) Clinical features. — Although lipomas occur more fre- quently than any other genus of connective-tissue tumours, and may, in most instances, be diagnosed with absolute certainty, yet under some conditions they are very puzzling, and give rise to much difference of opinion. The sub- cutaneous species is rarely the source of doubtful diag- nosis, unless situated in the palm, the sole, or on the scalp. The intimate relation between the tumour and the overljdng skin, the absence of definite boundaries, and its dough-like consistence are usually sufficiently trust- 24 OONNEGTIVE- TISSUE TUMOURS worthy guides. When a Hpoma is connected with the periosteum of the femur, the tibia, or the fibula it simulates a sarcoma ; when embedded in a muscle the most divergent opinions are often expressed in regard to the nature of the tumour ; and a lipoma in the posterior triangle of the neck has been mistaken for an aneurysm of the subclavian artery. Reference has already been made to those large lipomas which arise in the subperitoneal tissue, and the way in which they mimic the signs of ovarian tumours. A lipoma in the groin is occasionally mistaken for an irreducible epiplocele. Especial attention must be drawn to supposed fatty tumours situated in the middle line of the back: in most cases these are abnormal masses of fat overlying the sacs of spinse bifidse. Incautious surgeons, in operating upon such tumours, have unexpectedly opened the dura mater. Treatment. — Solitary subcutaneous lipomas should, as a general rule, be removed. When very many tumours are present (ten or twenty) it is not customary to interfere with them, for when multiple they rarely attain uncomfortable or dangerous proportions. It occasionally happens with 'multiple (and also with solitary) lipomas, that one or other becomes irritated by some part of the dress, such as petticoat bands, braces, etc., or in some particular employment followed by the individual. Such tumours should invariably be removed. The removal of a subcutaneous lipoma is one of the simplest proceedings in surgery, but the extirpation of a large subperitoneal fatty tumour is often attended with difficulty and grave danger. Berger, " Calcul salivaire et Hypertrophie de la Boule graisseuse de Bichat." — Gaz. des Hdp., 1883, Ivi. 1041. Bland-Sutton, J„ " On a Fatty Tumour of the Ascending Colon ; Enterectomy ; Recovery."— iajice^, 1900, i. 1437, Bonnot, E., " The Interscapular Gland."— /(wm. of Anat. and Phys,, xliii. 43. Cooper, Sir Astley, " Case of a Large Adipose Tumour successfully extir- pated." — Med.-Chir. Trans., 1821, xi. 440. Edmunds, W., " Fatty Tumour from the lA^."— Trans. Path. Soc, 1893, xliv. 151. Eve, F. S., "Large Congenital Lipoma situated between the Abdominal Muscles and Vasciie."— Trans. Path. Soc, 1886, xxxix. 295. Forster, J. Cooper, " Fibro-Fatty Tumour of the Abdomen, weighing fifty-five pounds." — Trans. Path. Soc, 1868, xix. 246. EEFEEENGES 25 Gabryszewski, A., "Ueber Lipome des Samenstranges." — Deutsche Zeitschr.f. Chir., 1898, xlvii. 317. Gay, J., " Fatty Tumour on Sole of WooL"— Trans. Path. Sog., 1863, xiv. 243. Gowers, W. R., "Myo-Lipoma of Spinal Cord." — Trans. Path. Soc., 1876, xxvii. 19. Gussenbauer, C, "Ein Beitrag zur Kenntaiss der subpleuralen Lipome." — Arck.f. lilin. Chir., 1892, xliii. 822. Hatai, S. "On the Presence in Human Embryos of an Interscapular Gland corresponding to the so-called Hibernating Gland of Lower Mammals." — Anat. Anteiger, xxi. 369. Holt, B., "Fatty Pendulous Tumour of the Pharynx and Larynx." — Trans. Path. Soc., 1854, v. 123. Hutchinson, J., jun., " Lipomata in Hernial Regions." — Trans. Path. Soc, 1886, xxxvii. 451. Hutchinson, J., jun., " Fatty Hernise in Linea Alba." — Trans. Path. Soc, 1888, xxxix. 451. Knaggs, R. Lawford, " Enteric Intussusception caused by an Intestinal Lipoma ; Laparotomy ; Reduction ; Removal of Tumour ; Recovery. [Tv7o Unusual Cases of Intussusception.] " — Lancet, 1900, ii. 1573. Langemak, " Zur Kasuistik der Darmlipome." — Beit. %. klin. Chir. (Bruns), 1900, xxviii. 247. Lebert, " Traite d'Anatomie pathologique," Plate xvi.. Fig. 11, t. 1, 128. Lockwood, C. B., " Congenital Fatty Tumours of Sole of the Foot, and Fatty Tumour from Palm of Hand." — Trans. Path. Soc, 1886, xxxvii. 450. Obre, H., "Deposit of Fat within the Cervical Portion of the Vertebral Canal."— Tm«s. Path. Soc, 1850-51, iii. 248. Parona, F., " Caso di Lipoma all' Ovaja ed Ovidutto di Destra." — An7i,. di Ostet., Milano, 1891, xiii. 103, pi. 1. Pick, T. Pickering, " Enormous Fatty Tumour of the Abdomen." — Trans. Path. Soc, 1869, xx. 337. Pupovac, D., " Ueber seltene Localisationen von Fettgeschwiilsten." — Wie7i. Idin. JFooh., 1899, xii. 41. Ranke, " Ein Saugpolster in der menschlichen Backe." — Virchow's Arch. /. path. Anat., 1884, xcvii. 527. Shattock, S. G., " On Normal Tumour- like Formations of Fat in Man and the Lower Animals." — Proc Boy. Soc Med., Path. Sec, 1909, 207. Shattock, S. G., " A Large Laryngeal Lipoma of the Epiglottis and Base of the Tongue, with a collection of examples of Submucous Lipomata of the Intestines and Larynx." — Ibid., 285. Smith, T., "A Fibro-Muscular Polypus growing from the Uterus, and contain- ing a Cyst and a Small Fatty Tumour." — Trans. Path. Soc, 1861, xii. 148. Steinheil, "Ueber Lipome der Hand und Finger." — Beit. z. Idin. Chir., 1891, vii. 605. Stetten, D., "The Submucous Lipoma of the Gastro-Intestinal Tract." — Surgery, Gynceeology and Otstetrics, Chicago, 1909, ix. 156. Treves, Sir Frederick, "A Case of Lipoma of the Broad Ligament." — Trans. Clin. Soc, 1893, xxvi, 101. CHAPTER II CHONDROMAS (CARTILAGE TUMOURS) A CHONDROMA (or enchondroma) is a tumour composed of hyaline cartilage. Its tissue resembles histologically the bluish translucent cartilage of an epiphysis. This genus contains three species — (1) chondroma, (2) ecchondrosis, (3) loose cartilages in joints. 1. Chondromas. — This species in its most typical con- ditions occurs in long bones, and, as a rule, in relation with the epiphysial cartilages ; hence this tumour is most frequently observed in children and young adults. A single tumour may be present, but frequently many grow con- currently, especially on the long bones of the hand and feet. An exceptional example is represented in Fig. 12, but similar conditions have been described by Kast, Steudel, and Recklinghausen. Chondromas are always encapsuled, and often form deep hollows in the bones from which they arise. They are painless, grow slowly, and are firm to the touch. Fre- quently they undergo mucoid degeneration, then the softened area gives rise to fluctuation. This serves to distinguish them from osteomas, with which they are apt to be confounded clinically. A chondroma frequently calcifies, and sometimes ossifies (Fig. 13). The frequency of chondromas in those who suffered from rickets in early life may be due, as Virchow thought probable, to the existence of untransformed pieces of cartilage acting the part of tumour-germs. Such remnants of unossified cartilage are not difficult of demonstration in rickety bones (Fig. 14). A chondroma is a very benign tumour, and even when it grows into the skull may require a long time to destroy life, as a very rernarkable specimen in the museum of 26 CHONDROMAS 27 St. George's Hospital proves. It is a cartilage-tumonr which arose in the mesethmoid of a young woman, and then filled the nasal fossse and occupied both orbits, and dislocated the globes outwards ; it filled the antra, expanded the nasal bones, invaded the spheno-maxillary fossse and formed a large mound in the anterior fossa of #v\ Fig. 12. — MultiiDle diondromas. (The lad was stunted from rickets.) the skull, and almost reached the roof of the cranium. Its disruptive effects upon the facial bones were very extra- ordinary. In spite of this, the patient's health was but little disturbed ; she had no loss of intellect, and, it is believed, no paralysis. The course of the disease from its origin till the patient died was about six years. Although a chondroma invading the skull may require (as in the example just described) years to kill a patient 28 CONNECTIVE- TI88 UE TUMO UBS there is a situation in which in certain circumstances it will cause great distress and death — namely, in the pelvis. The effects which such tumours produce on the pelvic viscera are in some cases very remarkable. The specimen represented in section in Fig. 13 was obtained from a woman 21 years of age who, with a large chondroma in her pelvis, became pregnant. Delivery by natural means Fig. 13. — Pelvis occupied by a large, partially ossified chondroma, shown in sagittal section : from a woman 21 years of age who died from hysterectomy performed for obstructed labour at term. (Museum of University College, Zondon.) being impossible, hysterectomy was performed ; but the patient died. Apart from obstructing labour, the tumour had pressed on. the ureters and produced dilatation of both of them and sacculation of the kidneys. This unfortunate woman was known to have a tumour in her pelvis seven years previously to her tragic death. Pathological cartilage occurs in spindle-celled sarcomas (p. 55) ; also in tumours of the salivary glands, especially the parotid. Cartilage-containing tumours grow in the lachrymal CHONDROMAS 29 gland, in tendon sheaths (Walker), in the testis, and in the breast. 2. Ecchondroses. — These may be defined as small local overgrowths of cartilage. They are best studied along the edges of articular cartilages, the laryngeal cartilages, and the triangular cartilage of the nose. Ecchondroses are especially common in the knee-joint, and often in association with the condition termed rheu- matoid arthritis. They are frequent in the joints of indi- viduals who have passed the meridian of life, and they occur as small projecting prominences along the margins of the articular cartilage. Often the edge of the cartilage is pro- Fig. 14. — Condyles and epiphysial Hne of a rickety femur, -with a cartilage island. {Museum of the Middlesex Hospital!) duced into a raised prominent lip, the regularity of which is broken here and there by a sessile or a pedunculated nodule. When these nodules are examined, many of them present on their outer surface a convex outline, but on the inner aspect — that looking towards the joint — they are concave, the concavity being produced by friction during the movements of the joint, or by pressure when the parts are at rest. Occasionally erosion of the ecchondrosis may extend so deeply that by some extra movement ot the joint the pedicle is broken, and the detached nodule either falls as a loose body into the joint-cavity, or it may be retained in position by its attachments to the fibrous structures of the articulation. Laryngeal ecchondroses are by no means common; 30 G01!TNEGTIVE-T188UB TUMOURS they grow from the thyroid, cricoid, and occasionally the arytenoid cartilages, but very rarely from the semi-rings of the trachea. Paul Bruns collected fourteen cases of laryngeal chondromas ; of these, eight sprang from the cricoid, four from the thyroid, one from the arytenoid, and one from the epiglottis. Most of the ecchondroses of the cricoid cartilage sprang from the broad posterior plate. ' In many of the cases the inner and outer surfaces of the cricoid were involved, so that the tumour encroached upon the cavity of the larynx. Ecchondroses vary greatly in size ; some are scarcely larger than a pea, others may be ■ as big as a walnut. Morell Mackenzie described an example growing from the cricoid which attained the size of a bantam's egg ; in this instance the tumour extended downwards in front of the trachea. Small ecchondroses growing from the inner surfaces of the laryngeal cartilages are more dangerous than the larger examples springing from their outer surfaces. Ecchondroses, when projecting into the larynx, are covered with its mucous membrane ; they may be smooth or tuberculated, round or conical. In exceptional cases the overl3dng mucous membrane has been found ulcerated. Chondromas, when they project into the larynx, produce stridor and difficulty in breathing, and sometimes interfere with the movements of the vocal cords. When the tumours only involve the outer surfaces of the laryngeal cartilages they do not, as a rule, cause any inconvenience unless they are exceptionally large. Small outgrowths from the triangular cartilage of the nose are by no means uncommon ; they never attain a large size, and are always sessile. It is difficult to imagine that ecchondroses of the nasal cartilage could be a source of much inconvenience, but surgeons who study diseases of the nasal passages view them with disfavour. 3. Loose cartilages. — Bodies of various kinds are found loose in the cavities of large joints, but those to be considered under the head of chondromas, in addition to detached ecchondroses, are pieces of hyaline cartilage found hanging in the joint by narrow pedicles, or occupying depressions in the bone from which they are occasionally dislodged. Structurally they are composed of hyaline cartilage, and assume various CHONDROMAS 31 forms. Some appear' as flat discs, others are ovoid ; they may be perfectly smooth, or present an irregular, worm-eaten ap- pearance; and the majority are impregnated with calcareous particles. It is a remarkable fact that in many instances in which a loose cartilage has been found in one joint, a body identical in size and shape has been found in the corresponding joint of the opposite limb (Bowlby, Glutton, Weichselbaum). Loose cartilages may be single or multiple : several hundred may exist in one joint, and vary in size from a rape-seed to an almond. The origin of these cartilages is interesting. In large joints, such as those of the hip, knee, or shoulder, it is easy to demon- strate, in the recesses of the joint, near the spot where the synovial membrane becomes continuous with the margin of the articular cartilage, villous-like processes of the synovial membrane projecting into the joint. Under certain con- ditions, especially that known as rheumatoid arthritis, these villi become greatly enlarged and increase in number until the whole synovial membrane may be so covered with them as to become quite velvety in appearance. Structurally, these synovial villi consist of a reduplication of the serous mem- brane, and contain tufts of capillaries. As they enlarge, some of them undergo chonclrification, and this change may take place so extensively that a villous process is entirely converted into hyaline cartilage, which becomes the matrix for a deposit of lime salts. As these nodules of cartilage are merely sus- tained by narrow pedicles, the nodules may be detached either by their mere weight, by undue movement of the joint, or from axial rotation, and, tumbling into the joint, give rise to all the inconveniences characteristic of a loose body. Specimens occasionally come to hand in which cartilaginous bodies of this description may be found sessile among the fringes, or hanging on good pedicles, or with stalks so thin that they appear to be on the eve of detachment. Occasionally these overgrown synovial villi, instead of chondrifying, are converted into oval bodies which, on micro- scopical examination, present a central cavity surrounded by a laminated structureless substance. To the naked eye many of these oval bodies resemble cartilage, and it is only on microscopical examination that it is possible to distinguish 32 CONNECTIVE-TISSUE TUMOURS between them ; many are infiltrated with calcareous granules. These oval bodies are present, in some cases, in great number. On one occasion I counted 1,532 which were removed from the shoulder-joint. Bodies of this description occur not only in joints, but in compound ganglia and bursse. A good physiological type for the loose cartilaginous bodies which infest joints is furnished by the temporo-mandibular joint of the skate. A recess communicating with this articular cavity usually contains a collection of smooth cartilaginous bodies, in contour and size like melon-seeds. Treatment. — The operative treatment of chondromas has been greatly simplified since surgeons have appreciated the fact that these tumours, when growing in relation with bones, are distinctly encapsuled. Now, when it is necessary to inter- fere with a chondroma, even in cases where several tumours are present, it has become customary to incise the capsule and shell out the cartilage. In most instances this simple method is successful. Exceptionally, however, cases come under observation which demand more serious measures. When the cartilage-tumours are very numerous on the bones of the hand, the fingers are so crippled and useless that amputation becomes necessary. Fortunately, such severe treatment is very rarely needed. In the case of loose bodies in joints it is the usual practice, when the pieces of cartilage are in the habit of getting between the opposed surfaces of the joint, to open the synovial cavity and remove the loose body or bodies. When this manoeuvre is conducted with proper care it is highly successful. When the loose body is lodged in a sacculus, it is in a measure isolated from the general cavity of the joint, and does not call for inter- ference. The smaller bodies, which, like mice, slip in and out of the recesses of a complex joint, are more likely to give trouble than those larger pieces of cartilage, sometimes as big as chest- nuts, which the patients can grasp with their fingers and push in and out of the great cul-de-sac above the patella almost as readily as a marble may be manipulated under a tablecloth. Bowlby, A. A., "Rare Forms of Loose Bodies from the Knee-Joints." — Trans. Path. Soc, 1888, xxxix. 281. Bruus, Paul, " Enchondrom des Kehlkopfs." — Beit. z. Jdin. Cliir. (Bruns), 1888, iii. 347. BEFEBENGES 33 Glutton, H. H., "Symmetrical Loose Bodies from Two Knee-Joints." — Trails. Path. Soc„ 1888, xxxix. 284. Kast und von Recklinghausen, " Ein Fall von Enchondrom mit ungevvohnlicher Multiplication." — Virchow's Arch./, imtli. Anat., 1889, cxviii. 1. Mackenzie, Morell, " Fibroid Degerieration of the Cartilages of the Larynx." —Trans. Path. Soc, 1870, xxi. 58. Steudel, " Multiple Encliondrome der Knochen in Verbindung mit venosen Angiomen der Weichteile." — Beit, z. hlin. Chlr. (Bruns), 1892, viii. 503. Walker, R. R., '• A Case of Endochondroma of the Tendon-Sheath of a Finger." — Clin. Jonrn., 1908, 240. Weichselbaum, A., "Zur Genesis der Gelenkkorper." — Virchow's Arch. f. path. Anat., 1873, Ivii. 127. CHAPTER III OSTEOMAS (OSSEOUS TUMOURS) An osteoma may be defined as an ossifying chondroma. The genus contains two species: 1, compact osteomas; 2, can- cellous osteomas. 1. Compact osteomas. — These occur as sessile tumours on the parietal and frontal bones ; in the frontal sinus, roof of Fig. 15. — Osteoma of mandible. {St. George'' s Hospital JItcseicm.) the orbit, walls of the external auditory meatus, mastoid process, and angle of the mandible (Fig. 15). They are com- posed of tissue as dense and as hard as ivory, and are fre- quently called "ivory exostoses." Those which arise in the frontal sinus and orbit are very remarkable tumours, and may attain large proportions (Figs. 16, 17, and 18). Many large tumours removed from the maxilla and described as exostoses were large odontomes (see Chapter xxi.). Large osteomas of the facial bones sometimes produce hideous deformity, and when they grow from the bones forming the rim of the orbit occasionally destroy the eyeball. The clinical histories of some of these cases are very remark- 34 OSTEOMAS 35 able ; for example, a man came under Lediard's observation with a large osteoma protruding from the orbit (Fig. 16). The patient, a sailor, stated that the tumour was noticed at birth, when it seemed scarcely larger than a pea ; it slowly increased in size, and when he was 9 years old it destroyed the eye- ball. When he was 25 years of age the skin of the eyelid sloughed. Eight years later the tumour fell out of the orbit. The spontaneous detachment of an osteoma in this way is Fig. 16. — Sailor with a large osteoma growing from the orbit. (From a icater- colonr sketch in the Iltiseum of tlie Royal College of Surgeons.) due to necrosis of the tumour, and is parallel to the shedding of the antlers in the stag. Osteomas of the orbit which have resisted the efforts of surgeons to remove them have, years after such operations, fallen of their own accord. The large and exceedingly hard ivory-like tumours which grow in the frontal sinuses are uncommon. An admirable example figured by Baillie, and preserved in the museum of the Royal College of Surgeons, is unfortunately without history (Figs. 17 and 18). Osteomas of this kind arise occasionally in the frontal sinuses of oxen, and form huge irregular lobulated masses, 36 C0NNBGTIVE-TI88UE TUMOURS sometimes weighing as much as sixteen pounds. Similar tumours grow from the petrosal and encroach upon the Fig. 17. — Osteoma in the left frontal sinus (anterior view). cranial cavity ; some of these have been reported in veterinary literature as ossified brains ! Osteomas at the margins of the external auditory meatus have been especially studied because they are apt to obstruct the meatus and cause deafness ; when both meatuses are Fig. 18. — Osteoma in the left frontal sinus (seen from below). {Mksckiii of the Royal College of Surgeons.) affected — and this is not rare — absolute deafness may result. It is a curious fact that osteomas at the margin of the audi- tory meatus have been observed in many different races of OSTEOMAS 37 men. Professor Sir William Turner has drawn attention to observations of Seligmann, VVelcker, and Barnard Davis, and added some of his own, concerning the presence of such exostoses in certain deformed skulls described as Titicaca's Huanaka's, and Aymara's. Also in skulls from the Marquesas Islands, Sandwich Islands, Chatham Island, and New Zealand. It is not surprising that osteomas should arise from the walls of the external auditory meatus when we remember the number of centres by which the periotic cartilage is transformed into bone, and the various ossific elements that come into relation with each other at this meatus. 2. Cancellous osteomas. — These tumours in structure resemble the cancellous tissue of bone, and are soft in com- parison with the preceding species. They usually possess a thick covering of hyaline cartilage, and when growing at the distal end of the radius, or tibia, present a series of deep channels for the passage of tendons. Occasionally an osteoma is pedunculated ; more frequently it has a broad base. Osteo- mas, whether sessile or stalked, usually grow slowly, but in the course of years they sometimes attain large proportions. They are innocent tumours, but occasionally imperil life by mechanically interfering with the function of vital organs. Reid described a case in which an osteoma grew from the posterior surface of the odontoid process and projected into the neural canal to the extent of 8 mm., compressing the spinal cord with fatal effect. Although in themselves pain- less, osteomas sometimes induce pain by pressing on nerve trunks in their vicinity. Often an osteoma is quite harmless (Fig. 19). Multiple cartilage-tipped osteomas are most frequent on the long bones of the arms and forearms, thighs and legs, and are often congenital, hereditary, and; so far as position is con- cerned, fairly symmetrical. Otto Weber recorded a remark- able case of numerous symmetrical exostoses of the long bones of the upper and lower limbs, the ribs, and scapula in a man 25 years old. A chondro-sarcoma arose in the right hip-bone and attained enormous proportions. It perforated the left external iliac vein, and pieces of the tumour, detached as emboli, lodged in the pulmonary artery. Exostoses. — It has been customary to describe all kinds 38 CONNECTIVE-TISSUE TUMOURS of tumours composed of bone, or bone-like tissue, under the name of exostoses. The term exostosis should be limited to irregular outgrowths of bone to which the term tumour is not in any sense applicable. The various bony outgrowths classed as exostoses fall into three groups : — 1. Ossification of tendons at their attachments. 2. The subungual exostosis. 3. Calcification of inflammatory exudations. 1. Exostoses formed hy ossification of tendons at their attachments. — The lono- bones of a child at birth are smooth Fig. 19. — Cancellous osteoma of the scapula. {HCuseuni of the Eoyal College of Siirgeons.) in outline and almost cylindrical in shape ; the periosteum is relatively thick, and gives attachment to the muscles. On ex- amination of the long bones of an adult muscular man their shafts are found to be irregular, and present many asperities, such as the linea aspera, gluteal ridge (sometimes called the third trochanter), oblique lines, and the like. These ridges and lines, in the majority of instances, are the ossified inser- tions of muscles; occasionally they are so pronounced as OSTEOMAS 39 to be appreciable through the soft structures, and are then described clinically as exostoses. The two most frequent examples of this form of exostosis are the adductor tubercle of the femur and the tubercle on the first rib at the insertion of the scalenus anticus. Probably the most common exostosis is that which occurs in the tendon of insertion of the adduc- tor magnus (Fig. 20) : it usually assumes the form of a broad ledge of bone ; exceptionally it is stalked, and in rare cases surmounted by a bursa ; the walls of such burste are now Fig. 20. — Exostosis of the femur, produced by ossification of the tendon of the adductor magnus. {Museum of the Royal College of Surgeons.) and then furnished with villi, and loose bodies have been found in them (Orlov/ and Riethus). Care must be taken not to confound a su23racondyloid process of the humerus, or the occasional third trochanter of the femur, with exostoses. Localized outgrowths are very common on the facial bones, especially the nasal processes of the maxillfe, where they may be unilateral or bilateral (Fig. 21). The cause of these exostoses is obscure. Small irregular osseous pro- minences are fairly frequent along the alveolar borders of the maxilla and mandible. 40 CONNECTIVE- TI8S UE T UM UBS Exostoses of the maxilla have been observed in natives of the West Coast of Africa, and in all probability have Fig. 21. — Symmetrical exostoses of the nasal processes of the maxillag. {After Hutchinson.) originated the myth of the existence of horned men in this region. Interesting particulars relating to this ques- tion are furnished by Macalister and by Lamprej^ Strachan Fig. 22. — So-called horned men of the Ivory Coast. {Maclaud.) has observed them in the West Indian negro, and Dr. Maclaud, of the French Navy, met with them frequently in the natives of certain villages on the Ivory Coast, where OSTEOMAS 41 the disease is known as "goundou." These bony swelHngs may become so large as to obscure the patient's vision, and in order to see over the top of them he is obhged to bend his head down. 2. The suhungiial exostosis is a troublesome outgrowth from the ungual phalanx of the big toe; it makes its way through the bed of the nail, and peers out between the nail and the skin at the tip of the toe, near the inner side (Fig. 23) ; its appearance is so characteristic that it only requires to be once seen to be appre- ciated readily. It is rarely bigger than a cherry-stone. When the soft investing tissues are removed, the tumour appears as a low prominence of cancellous bone jutting from the dorsal surface of the terminal phalanx. These outgrowths are probably due to the pressure of ill-fitting boots, J 1 ij r ^ J -a ^ig- 23.-Big toe with a and should be ranked among milamma- subungual exostosis, tory productions. 3. Exostoses due to calcification of infiaminatory exucla,- tions scarcely require consideration in this work; there is reason to believe that some of the cases described as multiple exostoses were really examples of the strange and rare disease known as " myositis ossificans." Bony tumours are of fairly frequent occurrence in all vertebrates. Paul Gervais has published descriptions of many interesting specimens from fish. Perhaps the most striking example is furnished by the skeleton of the fish ch^etodon, in Avhich some of the bones are furnished with rounded bony tumours. The museum of the Royal College of Surgeons contains many loose bones with tumours, as well as the skeleton of the original fish sent by William Bell to John Hunter (Fig. 24). Single bones of chaatodon are common in osteological collections. Cuvier explained this by stating that they were brought home by travellers who had eaten the fish. On section the outline of the ray can be seen running through the tumour. Clinical characters. — Osteomas are easily recognized on account of their extreme hardness, and by being 42 CONNECTIVE-TISSUE TVMOtfRS localized to bones : tliey rarely cause pain, except when growing in the vicinity of and pressing upon the trunks of nerves. Osteomas growing from the Avails of the auditory meatus will occasionally interfere with hearing, and if they are bilateral, and completely block both meatuses, produce total deafness (Field). Large osteomas of the orbit and frontal bone distort the eyeball, and produce hideous de- Fig. 24. — Bell's specimen of chsetoclon with its tumours and large occipital crest. formity. In determining the characters and mode of attach- ment of an osteoma, especially in the limbs, the X-rays render valuable assistance. Treatment. — Osteomas, unless they interfere with nerves or with the movement of joints, or, as in the case of the facial bones, produce deformity or deafness, are rarely interfered with. In a patient under the writer's care, with a large intrapelvic osteoma, a process of the tumour pressed upon the great sciatic nerve as it issued from the pelvis : this offending process was exposed through an in- cision in the buttock, and removed by means of a chisel and mallet. Pedunculated osteomas may be easily removed with the help of stout forceps. The removal of an ivory- osteoma sometimes requires the most persevering efforts of the surgeon, aided by the best surgical cutlery. Exostoses near joints should not be interfered with, unless they OSTEOMAS 43 produce great inconvenience. It should also be remembered, in removing' osteomas, that the cancellous tissue of the bone from which they grow is opened. Osseous tumours of the cranial bones are often formid- able objects for the surgeon ; when they grow from the roof of the orbit or the frontal bone, they not infrequently extend as deeply into the cranial cavity as they project beyond it. The museum of St. George's Hospital contains a small ivory tumour which grew on the frontal bone of a man. Keate vainly endeavoured to remove it with trephine, saw, chisel, and mallet for nearly two hours. Potassa fusa and nitric acid were applied to the base, and in the course of years the tumour dropped off. Sub- ungual exostoses are often so painful that patients are glad to have them removed. Fig. 25. — Thickened occipital bone of a fish, with a man's face artificially carved upon it. Bell, William, " Description of a Species of Chffitodon, called by the Malays Ecan Bonna."— P/n7. Trans., 1793, Part i., p. 7. Bland-Sutton, J., " On an Exostosis from a Fish."— Trans. Path. Soc, 1888, xxxix. 472. Field, G. P., "Osseons Tumours of the Meatus." — " Manual of Diseases of the Ear," London, 1893. Gervais, Paul, Journ. clc Zool., 1875, iv. Hutchinson, Sir J., "Illustrations of Clinical Surgery," 1878, i. 11. Lamprey, J. J., " Horned Men in Africa : Further Particulars of their Existence."— 5H^. Med. Joiirn., 1887, ii. 1273. Lediard, Trans. OpUlial. Soc, 1883, iii. 23. Macalister, A., " Further Evidence as to the Existence of Horned Men in Africa." — Proc. Boy. Irish Acad., 1883, 2nd Series, iii. 771. McGavin, L. H., " A Case of Multiple Osteoma associated with Chondro-Sarcoma of the W^hs."— Trans. Path. Soc, 1902, liii. 356. Maclaud, " Goundou or Anaklire (Gros Nez)." — JBrit. Med. Journ., 1895, i. 1217. 44 CONNECTIVE-TISSUE TUMOVUS Orlow, L. W., "Die Exostosis Bursata unci ihre Entstehung-." — Devtsche Zdtschr. f. CMt., 1891, xxxi. 293. Held, J., " Case of Disease of the Spinal Cord, from an Exostosis of the Second Cervical Vertebra." — Lond. and Edin. Mooitldy Journ. of Med. Sci., 1843, iii. 194. Riethus, 0., "Exostosis Bursata mit freien Knorpelkorpern." — Beit. z. hUn. CUr., 1903, xxxvii. 639. Strachan, H., " Bony Overgrowths or Exostoses in the West Indian Negro."— Brit. Mod. Jmirn., 1894, i. 189. Turner, Sir William, " On Exostoses within the External Auditory Meatus." — Jovrn. of Anat. and Phys., 1879, xiii. 200. Weber, Otto, " Zur Geschichte des Enchondroms namenlich in Bezag auf dessen hereditares Vorkommen und secundare Verbreitung in inneren Organen darch Embolie." — Virchow's Arch. f. path. Anat., 1866, xxxv. 501. CHAPTEK IV MYELOMAS A MYELOMA is composed of tissue identical with the red marrow of young bone. These tumours were formerly called myeloid sarcomas. The genus contains a single species — myelomas. These Fig. 26. — Microscopic characters of a myeloma from the acromial end of the clavicle. tumours arise only in the cancellous tissue of bone. When fresh the cut surface of the tumour is deep red, and looks not unlike a piece of fresh liver, and is very vascular. Micro- scopically, this tissue abounds in large multinuclear cells (giant cells, myeloplaques) embedded among round and spindle cells. The giant cells are so numerous as to consti- tute the greater proportion of the tumour (Fig. 26). The distribution of myelomas is that of red marrow, but they exhibit a striking preference for certain bones ; the tibia 45 46 G0NNEGTIYE-TI8SVE TUM0UB8 is tlie favourite bone in the lower, and the radius in the upper Hrnb ; whilst so far as the bones of the head are concerned, they appear to be peculiar to the jaw-bones. I have never seen a myeloma in a vertebra. In the long bones they arise in the shaft of the bone immediately adjacent to the epi- physial junction (Fig. 27) ; and if the epiphysial cartilage be present it would seem to play the same neutral part to a myeloma as to a sarcoma. Fig. 27. — Lower end of a femiu- in longitudinal section, showing a myeloma. (From a girl aged 16 years.) In the lower limb myelomas have been observed in all the large bones, but they show a decided preference for the head of the tibia. The tibia is the seat of a myeloma five times more fre- quently than any other long bone, and it is five times commoner in its upper than in the lower end (Figs. 28, 29). In the radius the lower end is the favourite site, but myelomas of the upper end are not unknown (Figs. 30, 31). MYELOMAS 47 The same reversal applies to the fibula and the ulna, myelomas preferring the head of the fibula, but the lower end of the ulna ; but in both situations they are very rare. In the clavicle several examples have been recorded in the sternal end, and I have observed one at the acromial end, an excessively rare situation (Fig. 32). In the humerus the upper end of the bone is the usual Fig. 28. — Coronal section of the upper end of the tibia showing a myeloma in the outer tuberosity. (From a woman of 25 years.) site, but in the fernur it is the condyloid end. A myeloma is very rare in the patella (Fig. 33). In the mandible myelomas affect the body of the bone, but in the maxilla they prefer the alveolar border, and may sometimes remain in the early stage restricted to the premaxilla. Clinical characters. — These are, as a rule, sufficiently characteristic to ensure accurate diagnosis. The patients are 48 CONNECTIVE. TISSUE TUMOURS young, rarely above 25 years of age; the tumour grows quite slowly, expands tlie bone, and thins the osseous capsule while expanding it until the bony shell is so thin that it crepitates when pressed by the finger (egg-shell crackling). Here and there the myelomatous tissue perforates the capsule and markedly pulsates synchronously with the cardiac systole. Myelomas do not infect lymph-glands, nor disseminate. Fig. 29.— Coronal section of the lower ends of the tibia and fibula, with the astragalus; a myeloma occupies the lower end of the tibia. (From a woman aged 23 years.) Fig. 30. — A myeloma of the upper end of the radius ; from a man of 28 years. (Museum, St. Thomas's Sosjntal.) Treatment. — When the patient comes under observation be- fore the tumour has perforated its capsule, it may be thoroughly extirpated without fear of recurrence. The manner of thorough extirpation varies with the situation of the tumour. In the upper limb, the lower extremities of the radius and ulna have been excised for myeloma, leaving an extremely useful hand. It is an important fact to remember that the lower third of the ulna may be excised alone, but when the radius is the affected bone it is an advantage to remove MYELOMAS 49 the corresponding section of the uhia. The upper third of the humerus, the inner half and the outer half of the clavicle have been resected for myeloma with excellent results. In the case RADIUS Fig. 31. — Myeloma of the lower eud of the radius. (Maseuij/, St. Thomases Hospital.) of the jaws partial excision has been performed for myeloma with good consequences, but when the patient allows one of these tumours in the maxilla to fungate before seeking surgical aid, the marrow tissue will so invade the surrounding soft Fig. 32. — Myeloma of the acromial end of the clavicle ; from a woraan of 26 years. {Museum, Royal College of Surgeons.) parts that complete extirpation is a chance event and recur- rence is probable. In the lower limb the best method of dealing with myelomas is not so certain. For those in the lower end of the femur, amputation is necessary. This method has also E 50 CONNECTIVE-TISSUE TUMOURS been employed for the patella by Robert Jones. Excision has been successfully employed for myelomas in the head of the tibia (Morton), and in this situation a milder method — enucleation, first suggested and practised by Paget — has given excellent results. A close study of myelomas indicates that they differ histologically, pathologically, and clinically from sarcomas, with which they have hitherto been grouped. They are rare tumours, and a careful perusal of periodical literature and hospital reports makes me think that at each of the eleven large general hospitals in London one myeloma a year is above the average. The subjoined table represents the good results which attend the surgical treatment of this genus of tumours. •■ Age or Patient Nature Keporter. AND BoKK Affected. OF Operation. Result. Reference. Morris . . 28. Lower end of Resection of lower Free from recur- Trans. Clin. Soc, radius ends of radius rence 16 years X. 138 ; xiii. and ulna later 155 ; xxii. 367. Lucas . . 29. Lower end of Resection of lo^^ er Free from recur- Trans. Clin. Soc, ulna end of ulna rence 10 years later X. 135 ; xxii. 366. Bland-Sutton 2C. Acromial end Resection of outer No recurrence 17 Trans. Clin. Soc, of clavicle half of clavicle years later xxiv. 12. (See Fig. 82.) Glutton . . 28. Uppo.r end of Resection of upper Died 1 J years later Trans. Clin. Soc, radius fourth of radius of albuminuria. No recurrence xxvii. 86. {See Fig. 30.) Glutton . . 35. Head of Amputation No recurrence 10 Treves's "System tibia years later of Surgery," i. 915. Bland-Sutton Preniaxilla Kxcision of the premaxilla No recurrence 3 years later Unpublished. Bland-Sutton 23. Lower end of Am putation No recurrence 4 Unpublished. (See tibia through middle of the leg years later Fig. 29.) Jones (Robert) 20. Patella Amputation No recurrence 3 years later Trans. Path. Soc, xlvi. 143. (See Fig. 33.) It is fair to assume that the remarkable case in which Mott in 1828 excised the sternal two-thirds of the clavicle for what he called, in the terms of his day, " an osteo- sarcoma," in a lad 18 years of age, was in all probability a myeloma. The boy survived the operation fifty years (Porcher). One of the difficulties connected with the treatment of a myeloma is the doubtful character of the diagnosis in some instances. A myeloma at the lower end of the MYELOMAS 51 radius is rarely missed, but in other long bones a tumour of this kind is simulated by tuberculous disease, the common species of sarcomas, gumma, and (rarely) echino- coccus disease. In well-marked examples the thinned and expanded bone furnishes the classic egg-shell or parch- rig. 33.— Myeloma of the patella ; from a giil of 20 years. {Museum, Roijal College of Surgeons) ment-like crackling, which is a clinical feature of great value, and was especially marked in the myeloma at the acromial end of the clavicle (Fig. 32). Butlin, Henry T., and Colby, F. E. A., " On Sarcoma of the Bones of the Thigh and Leg." — St. Bart's Hos2). Bejrts., 1895, xxxi. 31. Hinds, " Case of Myeloid Sarcoma of the Femur treated by Scraping." — Brit. Med. Joicrn., 1898, i. 555. Porcher, F. Peyre, " Post-mortem Dissection of the Kegion of the Clavicle, this bone having been removed for Osteo-Sarcoma by Dr. Valentine Mott, of New York, in 1828, when the subject was in his 19th year, and 54 years before his death." — Avier. Journ. Med. Set., 1883, Ixxxv. 146. CHAPTER V SARCOMAS; THEIR HISTOLOGIC CHARACTERS The term sarcoma is applied to any connective-tissue tumour which exhibits malignant characters. As a matter of fact, almost any kind of connective tissue — fat, bone, cartilage, and even striated- muscle tissue — may occur in sarcomas, but, as a rule, the greater part of the tumour consists of imma- ture connective tissue in which cells preponderate over the intracellular tissue. The species is determined according to the prevailing type of cell : thus we have round-celled and spindle-celled sarcomas ; some contain pigment, and are known as melano-sarcomas. Of each there are one or more varieties, which have received qualif3dng names, such as lympho-sarcoma, m3^o-sarcoma, chondro-sarcoma, and the like. 1. Round -celled sarcomas. — This species is of very simple construction, and consists of round cells with very little intercellular substance. Each cell contains a large round vesicular nucleus, and a small proportion of protoplasm ; the nuclei are always conspicuous objects in stained sections. Blood-vessels are abundant, often appearing as mere channels between the cells. Lymphatics are absent. Round-celled sarcomas grow very rapidly, infiltrate surrounding tissues, recur quickly after removal, and give rise to secondary dej)osits, especially in the lungs. There is a variety, known as the large round-celled sarcoma, in which the cells are of unequal size ; some of them contain two or more nuclei ; a few are multinuclear and resemble myeloid cells. The round-celled sarcoma is the most generalized tumour that affects the human body ; it may occur in any tissue, osseous, muscular, nervous, thymic, ovarian, or testicular, and even in the delicate sustentacular framework of the retina. It attacks the body at all periods of life, from the foetus in utero 52 SARCOMAS 53 and the child just born, up to the extreme limits of age ; and arises in vestigial organs, as well as in those which are in the full exercise of their functions, such as the kidney or the parotid gland. 2. Lympho-sarcomas consist of cells identical with those of the round-celled species, but the cells are contained in delicate meshes: the tissue resembles that of lymph-glands (Fig. 34), hence the origin of the term lympho-sarcoma. These tumours must not be confounded with simple (irritative) en- largement of lymph-glands, nor with the general overgrowth of lymphadenoid tissue associated with leukaemia or lymph- Fig. 34. — Microscopic characters of a lympho-sarcoma from the mediastiuum. adenoma (Hodgkin's disease). The lympho-sarcomas exhibit a very characteristic structure, occur as a rule in very definite situations, and have somewhat special clinical features. These tumours occur in the superior mediastinum, in the subpleural and subperitoneal connective tissue, at the base of the tongue, in the larynx, in the tonsil, and in the testis. Fortunately, sarcomas of this species are rare, for they are excessively malignant. 3. Spindle-celled sarcomas. — The cells in this species vary much in size, but they all agree in being oat-shaped or fusiform (Figs. 35, 36). The cells tend to run in bundles, which take different directions, so that in sections of the tumour seen under the microscope some bundles will have the cells cut in the direction of their length, and others at 54 CONNECTIVE -TISSUE TUMOURS right angles. This must be borne in mind, or an incorrect opinion will be formed as to the nature of the tumour. Fig. 35. — Microscopic characters of a small spindle-celled sarcoma from a metacarpal bone. In some sarcomas the cells are so slender and contain so little protoplasm that they appear to consist of merely a nucleus Fig. 36. — Section of a spindle-celled sarcoma from the first phalanx of the thumb. {Sighly magnified,') and cell-processes. In others the cells are large, fusiform, and rich in protoplasm, and resemble the cells of young unstriped SARCOMAS 55 muscle. Occasionally these spindle cells are transversely striped like young striated muscle-fibre. Another peculiarity of spindle-celled sarcomas is the frequent presence of tracts of immature hyaline cartilage ; indeed, in many instances this tissue constitutes so large a proportion of the tumours that they are described as chondro- mas ; the cartilage is sometimes calcified, and even ossified. It may seem strange to associate tumours containing striped cells and cartilage with sarcomas, but the correctness of the classification is demonstrated by the fact that such tumours are apt to recur after removal, and in some of the cases in which the primary and recurrent tumours have been carefully examined the primary tumour has contained cartilage, or muscle, whilst the recurrent mass has shown no evidence of these tissues, but has conformed to the structure of a pure spindle-celled or a round-celled sarcoma. In order, therefore, to indicate the nature of such composite sarcomas, they will be referred to as myo-sarcomas (rhabdo-rayomas) and chondro-sarcomas. Spindle-celled sarcomas often contain round and even multinuclear cells. Myo - sarcomas. — It is a remarkable fact, considering the large amount of striped-muscle tissue existing in the body, that tumours composed of or containing this tissue do not arise in connexion with the voluntary muscles, but make their appearance in such unexpected situations as the kidney, testis, neck of the uterus, parotid gland, and in organs and tissues which, under normal conditions, do not contain muscle- cells of the striped variety. Shattock has published an account of four examples Avhich grew in the bladder of children. The tumour in each instance assumed the polypoid form so common with sarcomas growing in the vagina of infants. M3^o-sarcomas of the testis have been mainly observed in children (Hulke, Neumann, Ribbert). Prudden has found cells with the transverse markings in a tumour from the angle of the mandible of a boy 7 years of age ; other examples connected with the periosteum have been reported by Zenker and Bayer, who found them in the orbit. Targett found one on the scapula of a child 6 months old ; and Marchand has described one 56 CONNECTIVE-TISSUE TUMOURS which grew from the ischial tuberosity of a boy 4 years of age. Pernice has recorded in detail a remarkable example connected with the neck of the uterus. The tumour con- tained a large number of transversely striated spindles (Figs. 37, 38). The tumour was removed, but quickly Fig. 37. — Racemose sarcoma of the neck of the uterus, {^fter Fernice.) recurred ; it was removed a second time, but reappeared and speedily caused death. A careful examination of the recurrent tumour showed it to consist of spindle cells, but no striation could be detected. Grape-like (racemose) sarcoma of the neek of the uterus. — Pernice's specimen, to which reference has already been made, belongs to a rare variety of sarcoma, of which about a dozen carefully observed examples have been described since Spiegelberg drew attention to this disease 8ABG0MAS 57 in 1879 (Whitridge Williams). Curtis has described an example which occurred in an infant a year old, and has collected the literature. In some of the specimens the grape-like bodies are covered with columnar epithelium, the bulk of the grape consisting of oedematous spindle- and round-celled sarcomatous tissue. In an example which I had an opportunity of examining, the grape-like bodies were hollow, and lined Fig. 38. — Microscopic characters of the uterine sarcoma shown in Fig. 37, containing muscle-cells. (Pernice.) with columnar epithelium, and led me to regard them as dilated glands in the cervical endometrium involved in a sarcoma. This form of tumour has been observed exclusively in girls and young women: it is very malignant, recurs locally, invades the uterus in the late stages, and gives rise to metastases. Sarcomas of the subperitoneal tissue. — Very large spindle-celled sarcomas are occasionally found in the belly and pelvis, arising in the subperitoneal connective 58 CONNECTIVE-TISSUE TUMOURS tissue. These tumours present some peculiar features. In tlae first place, the}^ are nearly always globular, and not infrequently resemble a football in shape and in size. They have been observed in the neighbourhood of the kidney, and in some instances this organ occupies a recess in the tumour. Retroperitoneal sarcomas of this kind often have the adjective perirenal applied to them. I have removed a tumour of this character as big as a coco- nut from between the layers of the mesentery. The patient was a "woman aged 25. The museum of McGill College contains a large globular tumour of this kind, weighing eight pounds, which was removed by Shepherd in 1897 from the mesentery of a man aged 28 years ; eight feet of small intestine was removed at the same time. The man was alive in 1900. Many of the tumours reported as " m3^oma of the broad ligament " are |)robably large, slowly growing spindle-celled tumours. They appear to be the least malignant of all the varieties of sarcomas, and are extremely rare. Many of the reported cases weighed upwards of thirty pounds. The genus sarcoma is certainly very heterogeneous and unsatisfactory, and will continue so until the cause of malignant connective-tissue tumours is discovered. The difficulty in regard to jfibromas, myxomas, and myomas has long been recognized ; for example, fibromas, or tumours composed of fibrous tissue, were regarded as common, but careful histologic research has shown them to be very rare. The tumours of the uterus known as myomas and ^fibro-myomas were formerly regarded as fibromas: traces of this belief still linger in the term " uterine fibroids." Many tumours now called spindle-celled sarcomas were, a few years ago, called " recurring fibroids." The difficulty of distinguishing between a myoma, a slowly growing spindle-celled sarcoma, and a fibroma is well known to skilled histologists. Myxomas. — These are tumours composed of tissue identical with the jelly-like substance which exists in the umbilical cord. Here we have to deal with a difficulty, because there is a very great tendency in many connec- tive-tissue tumours to degenerate into this soft gelatinous MYOMAS 59 or myxomatous tissue and become as diffluent as the vitreous body in the eyeball. The common nasal polypus furnishes an excellent example of this tissue ; it consists of cells with long, slender processes interlacing with those of adjacent cells and ramifying in a structureless, unstainable, diffluent mass, the whole being bounded by a thin layer of mucous membrane covered with columnar ciliated epithelium. Nasal polypi may be regarded as pendulous processes of (Edematous mucous membrane. It would be convenient and justifiable to deprive myxomas of even the rank of species among tumours. The heart is of all the organs of the body the least liable to tumours, primary or secondary, yet the few examples of primary tumours which have been observed in it are described by the reporters as fibromas, or myxomas, or fibro-myxomas. The chief cases have been collected and the clinical signs analysed by Pavlowsky. Myomas, or tumours composed of unstriped-muscle fibre, are very rare, and are met with exclusively in organs containing this tissue, e.g. the oesophagus, stomach, duo- denum, bladder, and uterus. Attention has already been directed to the difficulty of determining between the fusiform cells of sarcomas and unstriped-muscle fibre. This difficulty is increased by the fact that many malignant tumours composed of spindle cells (sarcomas) contain tracts of cells which pre- sent a transverse striation such as is seen in voluntary muscle in its embryonic stage ; but it is remarkable that cells with the transverse striation occur in situations where voluntary muscle is not found normally. It is also a fact that tumours consisting of mature striated (volun- tary) muscle-fibre have not been observed. Much caution needs to be exercised before deciding that a tumour is a myoma; formerly many of the spindle-celled sarcomas of the choroid were regarded as myomas arising in the ciliary muscle. It is also extremely probable that many of the tumours described as myomas from the oesophagus (Hilton Fagge), stomach, duodenum, bladder (Parker, Terrier, and Hartmann), and vagina were sarcomas. 60 CONNECTIVE -TISSUE TUMOURS Dermatologists are familiar with small tumours ot the skin, which are occasionally multiple, and consist of smooth muscle-fibres. Such myomas may arise from the arrectores pili. Marc found one on the skin of the occiput of an infant which had a diameter of 3 '5 cm., and I re- moved one from the scrotum of a boy a few months old. In two instances I have removed tumours from the stomach which were regarded by an experienced patho- logist (Foulerton) as leio-myomas. In one instance the tumour was as big as an almond ; the other, as large as an orange, projected from the serous coat at the great cul- de-sac. The clinical course in each instance justified the microscopic characters as to benignity. The common situation for tumours containing unstriped muscle-fibres is the uterus (see Fibroids). Bland- Sutton, J., " A Tumour of the Mesometrium weighing twenty-two Tponnds."— Trans. Obstet. Soc, 1900, xli. 298. Clarke, J. Jackson, "A Large Fibroma of the Small Omentum." — Trans. Path. Soc, 1892, xliii. 60. Curtis, H. J., " Grape-like Sarcoma of the Cervix Uteri." — Trans. Oistet. Soc, xlv. 320. Fagge, C. Hilton, "Case of Myoma of the CEsophagus." — Trans. Path. Soc, 1895, xxvi. 94. Hulke, J. W., and Adams, W., " Tumour of the Testicle from a Young Child." —Trans. Path. Soc, 1860, xi. 162. Marc, Serg., "Ein Fall von Leiomyoma subcutaneum congenitum nebst einigen Notizen zur Statistik der Geschwiilste bei Kindern." — Virchow's Arch.f. path. Anat., cxxv. 543. Marchand, Felix, " Ueber eine Geschwulst aus quergestreiften Muskelfasern mit ungewohnlichem Gehalte an Glykogen, nebst Bemerkungen iiber das Glykogen in einigen fotalen Geweben." — Virchow's Arch. f. path. Anat, 1885, c. 42. von Neiunann, Prof. E., "Ein Fall von Myoma striocellulare am Hoden." — Virchow's Arch. f. path. A^iat., 1886, ciii. 497. Parker, R. W., " Case of Tumour (Myoma) of the Female Bladder removed by the Galvano-Cautery through a Dilated Urethra combined with Supra- pubic Incision." — Trans. Clin. Soc, 1888, xxi. 58. Pavlowsky, "Beitrag zum Studium der Symptom atologie der Neubildungen des Herzen-3. Poly pose Neubildungen des einken Vorhofs." — Berl. hlin. Woch., 1895, xxxii. 393. Peraice, Ludwig, "Ueber ein traubiges Myosarcoma striocellulare Uteri." — Virchow's Arch. f. path. A7iai,, 1888, cxiii. 46. Prudden, T. M., "Ehabdo-Myoma of the Parotid Gland." — Ajuer. Journ. Med. Soi., 1883, Ixxxv. 438. REFERENCES 61 Ribbert, " Beitrage zur Kenntniss der Rhabdomyome." — Yirchow's Arch. f. path. Anat., 1892, cxxx. 249. Shattock, S. G., " Rhabdo-Myoma of the Urinary Bladder."— Proc. Roy. Soc. Med. Path. Sec., 1909, p. 31. Shepherd, F. J., " Successful Removal of an Enormous Mesenteric Tumour and nearly eight feet of Intestine." — Brit. Med. Journ., 1897, ii. 966. Targett, J. H., " Congenital Myxo-Sarcoma of the Neck containing Striped Muscle-Cells."— Trrms. Path. Soc, 1892, xliii. 157. Terrier, F., et Hartmann, H., " Contribution k I'Etude des Myomes de la Vessie." —Rev. de Chir., 1895, xv. 181. Williams, Whitridge, " Contributions to the Histology and Histogenesis of Sarcoma of the Uterus." — Avier. Journ. of Ohstet., xxix. 721. Zenker, Konrad, "Zur Lehre von der Metastasenbildung der Sarcome." — Virchow's Arch. f. path. Anat., 1890, cxx. 68. CHAPTER \l SARCOMAS (Continued): THEIR GENERAL CHARACTERS Sarcomas are distinguished from the preceding genera of tumours in rarely possessing capsules, and when they do it is generally a spurious encapsulation depending on environment, as when they occur in the kidney, the eyeball, or the centre of a bone. It is lack of a capsule which permits them to in- filtrate surrounding tissues and favours dissemination. It will be convenient to devote this chapter to the consideration of the way in which sarcomas display their malignancy. Blood-supply of sarcomas. — The vascularity of sarcomas varies greatly ; in all, the circulation is mainly capillary. In the small round-celled species the vessels are so numerous as to cause distinct pulsation ; in the slow-growing spindle- celled varieties — especially those undergoing chondrification — the vessels are not numerous, and the tumours on section are yellowish white. It has already been pointed out, in describing the minute structure of sarcomas, that the walls of the vessels are very thin, and are often so attenuated as to resemble channels between the cells. This explains the frequency of htemorrhage within the soft and rapidly growing varieties. Repeated extravasations of blood will sometimes convert these tumours into cysts containing blood intermixed with sarcoma- tous cells. Tumours transformed in this way were formerly described as malignant blood- cysts. Although the vessels in a sarcoma are, in the main, capil- laries, nevertheless the -arteries supplying the tumour may be very large and numerous. When a sarcoma grows from the distal end of the femur and attains a large size, arteries sup- plying it from neighbouring muscular, periosteal, and articular trunks become important branches, and in such circumstances an incision into the tumour will be attended with alarminsf 62 SARCOMAS 63 haemorrhage. When attempts are made to dissect out such a tumour from the Hmb instead of adopting more radical measures, such as amputation, these enlarged vessels must not be forgotten, or they will intrude themselves upon the surgeon in a very unmistakable manner. Arteries which Fig. 39. — Section of lung, with nodules of sarcoma secondary to a chondi-if ying tumour of the testis. {Museum, JRoi/al College of Surgeons.) under ordinary conditions are almost inappreciable will, when nourishing a sarcoma, attain the dimensions of the radial or even larger trunks. Dissemination. — Sarcomas are liable to reproduce them- selves in distant organs, a phenomenon frequently referred to as metastasis. It is due to minute particles of the tumour growing into the venules ; these, becoming detached, are 64 CONNECTIVE-TISSUE TUMOURS transported by tlie current of blood to distant organs, where they become arrested by the capillaries, engraft themselves, and then grow into independent tumours. This dissemination takes place mainly through the veins, because, as already mentioned, sarcomas are devoid of lymphatics. The most common organ in which to find secondary sarcomas is the lung (Fig. 39), unless the primary growth is situated in the territory of the portal circulation, when they will be found in the liver. In very malignant examples, especially the small round-celled species, secondary deposits may form in any organ of the body ; they are always identical in structure with the primary tumour. Secondary deposits of sarcoma in the lungs may destroy life by mechanically obstructing the trachea and bronchi. I have known a nodule to slough and find its way into the trachea, and when expelled by coughing it be- came impacted between the vocal cords and suffocated the patient, a girl of 19 years. In this instance the primary tumour was a periosteal sarcoma of the femur, for which am- putation had been performed several months before. Infiltrating properties of sarcomas. — The tendency to extensive infiltration of the planes of connective tissue adja- cent to the tumour is not peculiar to sarcomas, for it is an obvious character of carcinoma. This property of sarcomas may be studied in a marked manner in the case of mediastinal lympho-sarcomas. These tumours grow rapidly, enveloping the trachea and bronchi, the aorta and other large vessels, the oesophagus, and large nerve-trunks. The tumour extends alonof the branches of the bronchi, and invades the interlobu- lar connective tissue at the roots of the lungs. When the tumour starts in the superior mediastinum it descends along the big vessels and invests the pericardium. It may even creep along the sheaths of the vessels to the heart and infil- trate its substance, and nodules of the tumour may project into the cavity of the auricles. Processes of the tumour may find their way along the sheaths of the big vessels and appear in the posterior triangles of the neck. The relation of a mediastinal lympho-sarcoma to the adjacent structures is interesting. For instance, the large arterial trunks, though embedded in the tumour, are not as a rule damaged by it. The aorta may be so compressed by the Sarcomas eh tumour as to produce a murmur ; the thin-walled veins are early compressed, and interference with the venous circula- tion is a marked feature. In some of the cases infiltration of the walls of the veins takes place, and processes of the tumour project into their channels. The bronchi are very liable to be damaged by a lympho- sarcoma, for the tumour moulds itself round these tubes, and by pressure causes them to be narrowed; apart from this effect, the tissues proper of the tubes become eroded and destroyed. These changes not only induce difficulty in re- spiration by restricting the admission of air, but the com- pression of the vessels accompanying the bronchi leads to changes in the nutrition of the pulmonary tissue, which end in pneumonia, gangrene, and death. The important nerves traversing the mediastinum, the vagus and phrenic nerves especially, are often involved in the tumour, but their sheaths are rarely invaded by the cells ; in some instances the left recurrent laryngeal nerve is compressed sufficiently to produce severe laryngeal spasms and even paralysis of the muscles supplied by it. The oesophagus becomes squeezed by an intrathoracic lympho-sarcoma, but dysphagia is not so prominent a symptom as in many cases of intrathoracic aneurysm. The oesophagus may be invaded and even perforated ; when this happens, ulceration and sloughing produce a cavity in the tumour, and may even broach the aorta (Hale White). It is a somewhat remarkable feature of lympho-sarcomas that they extend to and enclose neighbouring lymph-glands without affecting them : it is by no means unusual in a section of a large mediastinal sarcoma to find bronchial lymph-glands fully charged with pigment embedded in the tumour (Fig. 40). Some writers are of opinion that lympho-sarcomas of the superior mediastinum arise in the thymus. This, of course, is possible, but it is very difficult of proof. The infiltrating power of sarcomas may be studied when they invade the sheath of a muscle. For instance, when a retinal sarcoma protrudes through the sclerotic and invades the orbit it sometimes makes its way into the sheaths of the recti, and converts them into masses resembling yellow wax. On microscopic examination the F ee GONNEGTIVE-TISSJJE TUM0VR8 various fasciculi will be found isolated by tbe cells of the sarcoma. Periosteal sarcomas often invade muscles, and this is easily comprehended when the intimate relations of muscles to periosteum are remembered. Burrowing tendencies of sarcomas. — All tumours in their growth tend to follow the lines of least resist- Fig. 40. — Portion of a mediastinal lympho-sarcoma, to show the manner in which the tumour extends along the bronchi and pulmonary vessels. ance, and thus enter into nooks and crannies in the most unexpected manner. Every surgeon knows how a sarcoma of the maxilla will send processes into the spheno- maxillary fossa and creep through the foramen rotundum, to appear in the cranial cavity. Sarcomas springing from the heads of the ribs or processes of the vertebrae have been known to extend through intervertebral foramina and com- press the cord, giving rise to fatal paraplegia (Fig. 54, p. 91). SARCOMAS 67 It is also remarkable what slender barriers will serve as checks to sarcomas. For example, it is no uncommon condition for one of these tumours springing from the periosteum near a joint to extend in all directions and envelop the synovial membrane, yet be hindered by it from invading the joint. ! Relation of sarcomas to veins. — It has long been recognized that when sarcomas become disseminated the secondary tumours occur in situations which indicate that the distribution has been effected by means of the veins. Attention has been drawn already to the tendency which seems inherent in most species of sarcomas to burrow ; this tendency comes out in a striking way when studied in connexion Avith veins. Perhaps the simplest form occurs in the eyeball. When a melanoma arises in the uveal tract, especially when the tumour is in close relation with the choroid, it remains for a period restricted to the interior of the globe, until it pro- duces such changes in the intra-ocular tension that the cornea sloughs and the growth protrudes externally. In many of these specimens, if the sclerotic be carefully examined in the situations where the vense vorticosse pierce it, small nodules of the tumour will be detected projecting through these openings, having made their way out by burrowing in the sheaths, and in some cases actually travelling along the lumina of the veins. The relations of sarcomas to veins come out strongly when these tumours affect bones. In some examples of periosteal sarcomas the medulla is invaded by processes of the tumour making their way along the veins traversing the Haversian canals. The converse of this is also true, for a central sarcoma will sometimes implicate the perios- teum by w^ay of the Haversian canals. It is well established that most examples of central sarcomas occur near the joint ends of bones, and yet it is exceptional to find the joints invaded. When joint in- vasion happens, it occurs late in the course of the disease, and then, in most cases, the tumour creeps in through the synovial membrane. This comparative immunity of joints is usually attributed to the articular cartilage acting 68 CONNECTIVE-TISSUE TUMOURS as neutral tissue ; but it appears rather to be due to the fact that the cartilage, unlike the compact tissue of bone, is not traversed by a multitude of narrow venous chan- nels. Extraordinary examples of the invasion of veins by sarcomas occur in the abdomen. In cases of renal sar- Inferior vena cava. Intravenous process of the sarcoma. Glands infected by sarcoma. J A sarcoma springing i^ from tlie ilium. Fig. 41. — Periosteal sarcoma of the ilium invading the inferior vena cava, {lliiseum, St. BarthoIo)neu-''s Hospital.) comas, processes of tumour will find their way into the renal vein, and thus enter the inferior vena cava. Periosteal sarcomas of the pelvic surface of the ilium are very liable to infiltrate the iliac veins and extend into the vena cava (Fig. 41). When processes from a sarcoma project into a vein, the circulating blood is apt to detach large frag- ments, and these become dangerous emboli. The mere presence of a sarcomatous outrunner in a vein SARCOMAS i 69 does not necessarily imply dissemination of the sarcoma, for very large intravenous processes may exist, and the lungs be free from any gross lesion of a sarcomatous nature. On the other hand, a very small invasion may lead to extensive infection of the lungs, especially if the protruding surface of the tumour be eroded by the blood- current. Dr. Pitt has described a case in Avhich a man with sar- coma of the thyroid gland died suddenly. At the post- mortem examination the cavities on the right side of the heart were found to contain fragments of growth embedded in clot ; on dissection it was ascertained that the sarcoma had ulcerated into the internal jugular vein. When a vein is invaded by a sarcoma, and discharges of emboli frequently occur, they easily traverse, when small, the right auricle and ventricle, but are too large to pass through the pulmonary capillaries; hence the small vessels in the lungs act as filters, and these arrested particles act as grafts, and grow into secondary nodules. Secondary changes. — Sarcomas are very prone to de- generative changes ; for instance, hsemorrhage is very apt to take place in those which grow quickly, producing spurious cysts. The tissues of the tumour are prone to liquefy, and myxomatous changes are very common. Calcification occurs in those which grow slowly, especially if connected with bone. When sarcomas grow rapidly and involve the skin, ulceration may occur and lead to profuse and oft-repeated haemorrhages, which not only exhaust the patient, but in many cases induce death. Occasionally considerable portions of a sarcoma will necrose, especially in very large tumours. In such cases a cavity forms in the sarcoma, and on cutting into it the fluid escapes, with large irregular pieces of the tumour, which are generally of a greyish- white colour. When necrosis occurs extensively in a large sarcoma it will sometimes check the course of the tumour in a very marked manner. Distribution. — As connective tissue occurs in every organ of the body, so sarcomas are ubiquitous, but they occur in some situations more commonly than in others. They frequently grow from subcutaneous tissue and fascia, 70 G0NNEGTIVE-TI88UE TUMOURS periosteum, the testicle and ovary. They are so rare as primary tumours of the liver, spleen, and bowel that it is not possible to write a general account of such tumours, from lack of material. As primary tumours of voluntary muscles, sarcomas are rare. They may be of the round- celled or the spindle- celled sj)ecies. For a time, at least, the tumour is limited by the sheath of the affected muscle. At first the tumour appears localized to a particular spot in the muscle, but it gradually extends until the whole belly of the muscle is involved and becomes transformed into an indurated mass. On section the muscle appears to be replaced by hard, tough, pale-grey material. When sections are examined under the microscope the' appear- ance is very striking, for each fasciculus is isolated from its neighbour by collections of cells characteristic of the sarcoma. As in sarcoma of other organs, haemorrhage is very liable to occur in the substance of the tumour, leading to the formation of cavities with ragged walls. Primary sarcomas have been recorded in the follow- ing muscles : rectus abdominis, peroneus longus, gracilis, tensor vaginae femoris, adductor brevis, sartorius, tibialis anticus, and the triceps. Four cases under my own notice occurred in the pectoralis major, the extensor carpi radialis, the adductor longus, and the vastus externus. The age-distribution of sarcoma of muscle is a wide one ; in the instances enumerated above, the youngest patient was 18, and the oldest 60 years. The disease shows a marked preference for the muscles of the lower limb. Extreme care is necessary to avoid mistaking a syphilitic ■ gumma in a muscle for a sarcoma. It is a curious fact that sarcoma of nerves should show the same preference for the lower limbs as in the case of muscles. In the majority of instances it is the great sciatic, or its branches, the popliteal, posterior tibial, peroneal, or the plantar nerves. In more than half the cases it is the trunk of the great sciatic which is attacked. Sarcoma of synovial membrane. — A primary sarcoma of a synovial membrane is a rare disease ; and it shows the same marked preference for this membrane in the lower 8ABG0MAS 71 limb as is the case with muscles and nerves. The tumours may be of the round- or the spindle-cell species, but some contain giant cells and cartilage. The disease may be diffuse, or so localized as to be a distinct tumour, and it rarely takes the form of pedunculated bodies. It attacks men and women equally, and the age of the patients varies from 20 to 35 years. The disease is of slow progress, and causes the patients very little incon- venience, as it does not interfere with the movements of the joint. The diagnosis is a matter of great difficulty, as the disease resembles a tuberculous affection of the joint more strongly than anything else. In Annandale's patient the disease was regarded as a myeloma, and in a patient under my own care the enlargement of the joint and the inter- ference with its mobility were attributed to loose bodies, and the operation was undertaken on this diagnosis. The disease lends itself to three kinds of operative treatment : — (a) Enucleation, when the disease is limited to a por- tion of the synovial membrane. Turner has successfully practised this treatment on the ankle-joint, and Howard Marsh on the knee. My patient was free from recurrence five years after the operation (Fig. 42). (6) Resection of the joint when the sarcoma is diffuse. (c) Amputation. This seems to be the best guarantee against recurrence, and is a method of treatment more par- ticularly resorted to when the sarcoma is diffuse. JuUiard and Descoeudres have reported an additional case, collected the records, and carefully summarized the facts relating to this rare disease. Primary sarcoma of bursae. — It is well known that burste are prone to undergo inflammatory changes, especially when situated in exposed situations, such as those which arise in relation with the patella, and it is a matter of common observation that a prepatellar bursa when chroni- cally irritated, as in housemaids and carpet-layers, will become almost solid : specimens illustrating this are common in pathological museums. There are a number of care- fully observed cases which show that a bursa may become the seat of sarcoma, and in which local recurrence followed 72 CONNECTIVE-TISSUE TUMOURS extirpation of the tumour. Sarcomatous bursse have been observed in connexion with the patella, the semimem- branosus sac at the knee-joint, and the subdeltoid bursa. The chief clinical signs on which a diagnosis may be founded would appear to be these : a chronically enlarged bursa takes on active growth, and becomes firmer in con- sistence, and this is accompanied by great enlargement of the veins in the skin overlvino' the bursa. Fig. i2. ^Pedunculated bodies removed from the knee ; the joint contained thii'ty-six such bodies. It must be remembered that prepatellar bursse in syphilitics sometimes rapidly solidify. The literature of sarcomas arising in bursal sacs has been collected by Adrian. It is characterized by great poverty. Sarcomas of the alimentary canal. — Although carci- noma is the prevailing type of malignant disease which attacks the alimentary canal from the oesophagus to the anus, cases of sarcoma have been observed and reported in sufficient numbers to enable their leading clinical features SARCOMAS 73 to be summarized. The disease arises in the submucous tissue, and may assume the form of a j^olypus, or infil- trate the wall of the canal, or project on the surface of the intestine in the form of plaques. All species of sarcomas have been observed. It is also noteworthy that sarcomas are more prone to attack those regions of the stomach and Pedicle. Abscess cavity. Tumour. Fig. 43.— Portion of jejunum in section ; a pedunculated tumour had invaginated the bowel and produced intestinal obstruction. (From a man 35 years of age.) intestines which are in a measure respected by carcinoma. Thus, in the stomach, sarcomas prefer the body of the organ, and they occur with greater frequency in the small than in the large intestine. In the small intestine the liability to the disease increases from duodenum to ileum. Sarcomas have been reported in the vermiform appendix. Secondary deposits appear to be most common in the liver. 74 CONNECTIVE- TI8S UE TUMOURS One of the most important clinical features Avhich distinguish sarcoma of the intestine, large or small, from carcinoma is its occurrence in the early years of life; many examples have been observed in children. The disease runs a more rapid course, causes more pain, and forms a much larger tumour than is the rule with car- cinoma. As a sarcoma often tends to become polypoid, the occurrence of intussusception is a frequent complica- Fig. 44. — A, Breast in section showing an ossifying sai'coma. * The nipple. B, The osseous element of the tumour. (Removed from a woman 73 years of age.) tion (Fig. 43). The results of operative treatment are unfavourable : rapid recurrence is the rule. Corner and Fairbank have collected and analysed the records of this disease in an admirable paper founded on a case under their care. The vagina is an uncommon situation for sarcomas, and here they exhibit unusual characters connected with age-distribution — for in children they have a great tend- SARCOMAS 75 ency to become polypoid, or they form flattened masses in tlie submucous layer. Occasionally the tumours may be multiple. Often the sarcoma interferes with the functions of the rectum and bladder. The literature of sarcoma of the vagina in infants has been collected by Power ; for adults, by W. Roger Williams and Gow. Sarcoma of the breast.— This gland is liable to be the seat of round- and spindle- celled sarcomas. They are rare tumours and grow slowly : some mammary sarcomas contain tracts of hyalin cartilage and bone. Examples of chondri- fying tumours have been described by Bowlby, Battle, Bruce- Clarke, Morton, and Gordon Watson. The only specimen which has come under my notice occurred in a multipara, aged 78 (Fig. 44). The tumour in this case was so hard that it had to be cut with a saw. A perusal of the reports of these cases shows that calci- fying and chondrifying tumours of the breast exhibit the worst features of sarcomas, namely, quick recurrence. Treatment. — This consists in the wide removal of the affected part, whenever possible, by means of the knife. The method of effecting this varies according to the seat of the disease, and the organ affected. In the ensuing chapters dealing with the distribution of these tumours, references will be made to the principles governing the surgical treatment applicable to each situation. There are many conditions, apart from the size of the tumour, which prevent its complete extirpation, such as its position in relation to vital organs, and generalization (metastasis) : when sarcomas do not permit of radical surgical treatment they are said to be inoperable. Much earnest investigation has been made with the hope of finding some means by which patients with inoperable sarcoma may be relieved, especially in the domain of serumtherapy. {See Chap, xxvii.) Adrian, " Ueber die von Schleimbeuteln ausgehenden NeubilduDgens." — Bruns, Beit., Bd. xxxviii. 459. Bland- Sutton, J., " An Ossifying Sarcoma of the Female Breast." This paper contains abstracts of five similar cases. — Arch, of Middx. Hosjj., 1910, xix. 98. Corner (and Fairbank), "Sarcoma of the Alimentary Canal."— Traws. FatJi. Soc, Ivi. 20. 76 CONNECTIVE-TISSUE TUMOURS Gow, St. BartJs Hasp. Repts., 1891, xxvii. 97. Griffiths, J., "Case of Villous Sarcoma of the Neck and Heart." — Trans. Path. Soc, 1888, xxxix. 311. JuUiard et Descoeudres, " Sarcoma primitive de la Synoviale du Genon." — Arch. Internat. de Cliir., Gand, 1904, p. 589. Lockwood, " A Case of Sarcoma of the Synovial Membrane of the Knee." — Trails. Clin. Soc, London, xxxv. 139. Marsh, Howard, "Primary Sarcoma of the Knee-Joint." — Lancet, 1898, ii. 1330. Pitt, G. Newton, " Sarcoma of Left Lobe of Thyroid, growing round oesophagus, and invading left internal jugular vein and left vagus. Ante-mortem clot on right side of heart, containing growth." — Trans. Path. Soc., 1887, xxxviii. 398. Power, D'Arcy, St. Part's Hasp. Repts., 1895, xxxi. 121, Salaman, R. N., " Sarcoma of the Stomach." — Trans. Path. Soc, 1904, Iv. 296. Turner, G. R., "Primary Sarcoma of the Synovial Membrane of the Ankle- Joint." — Trans. Clin. Soc, London, xxxv. 137. Williams, W. R., "Vaginal Tumours," 1904. CHAPTER VII SARCOMAS OF BONES These tumours arise in connexion with bones in two situations, either in the interior of a bone, or in the deeper (osteogenetic) layer of its periosteum : hence they are spoken of as central and periosteal sarcomas. 1. Central sarcomas may arise in the middle of the shaft, but more frequently they originate in the cancel- lous tissue near the ends of the long bones. Sarcomas arising in the diaphysis belong, as a rule, to the round- celled species. Those which grow at the extremities are generally spindle-celled, and contain a variable quantity of myeloid cells ; cartilage is sometimes present. They occur at any age, but are most frequent between 10 and 40, and are more common in the long bones of the lower than in those of the upper limb. When a tumour occuj)ies the centre of the diaphysis, its growth causes expansion of the osseous boundaries, and produces a rounded or spindle-shaped swelling, and the bone may become so thin that upon some slight exer- tion it breaks. In cases where the tumour affects the extremity of the bone it will, in young subjects, infiltrate the epiphysis, but it rarely transgresses the articular cartilage. Central sarcomas rarely affect the adjacent lymph- glands. In exceptional cases, especially with small round- celled sarcomas, the cells will make their way along the Haversian canals and form a tumour beneath the perios- teum. Central sarcomas lead to enlargement of the sur- rounding bone; hence when the soft tissues are removed by maceration a large bulb-like osseous mass is left. These specimens are common in pathological museums. In some cases this osseous capsule is so thin that the tissue of ' the tumour makes its way through, and as it is very 77 78 CONNEGTIVE- TISSUE TUMOURS vascular a strong rhythmical pulsation (accompanied by a bruit) is perceptible over the protruding portion. 2. Periosteal sarcomas. — These may be round-celled or spindle-celled, and are liable to the various metamorphoses and degenerations afi'ecting sarcomas generalh", but are more liable to calcification and ossification than central tumours. They occur earlier in life than those of the pre- ceding class, and are frequently associated with antecedent injury. They do not, as a rule, invade joints. When growing from the periosteum near the middle of the shaft, a sarcoma may be restricted to a portion of its circumference, or entirely surround it, producing a fusiform swelling. In such specimens the shaft of the bone traverses • the tumour, and beyond a slight amount of erosion, may be unaffected by it. In such a case, however, the medulla is sometimes infected by the cells making their way along the Haversian canals. Periosteal, like central, sarcomas have a greater predilection for the joint ends of the bone than for the central portion of its shaft. In size periosteal sarcomas vary greatly ; in exceptional cases they have been known to exceed a metre (40 inches) in circumference. Many become more or less ossified, the osseous matter taking the form of delicate spicules arranged at right angles to the shaft of the bone; sometimes it forms an irregular bony mesh, the spaces being filled with sarcomatous tissue. In some specimens the bone is greatly thickened in the parts related to the tumour. The exten- sive ossification associated with sarcoma of the periosteum is not a matter for surprise when we remember the bone- forming function of this tissue. The crystal-like sjDicules so frequently found probably represent ossification of the fibrous trabeculse which connect the periosteum with the compact tissue of the shaft ; as the periosteum is raised from the bone by the growing tumour, these trabeculse elongate and subsequently ossify. The femur. — This bone is very liable to sarcomas, especially the periosteal variety ; they are most frequently associated with its lower third, and invariably run a rapidly fatal course, the duration of life rarely exceeding eighteen months; often it is very much less. Usually they occur 8ABG0MA8 OF BONES 79 between the fifteenth and fortieth years. A sarcoma situated at the lower end of the femur often simulates disease of the knee very closely, and gives great difficulty in diagnosis ; also a sarcoma of the femur may invade the knee-joint and resemble a primary sarcoma of the Fig. 45. —An ossifying spindle -celled sarcoma of the femur : in transverse section. synovial membrane of that joint. The rapidity with which a periosteal sarcoma of the femur will destroy the patient, especially when it occurs in early life, is illustrated in the following case : — A man 24 years of age felt pain in his knee ; a month later it was found that a sarcoma occupied the lower end of his femur. Two months afterwards he came under my care, and the leg was promptly amputated. The tun^our, a periosteal sarcoma, had circum- 80 OONNEGTIVE-TISSTIE TUMOURS scribed the lower portion of the femur (Fig. 45). A few days after the operation, difficulty of breathing began to declare itself, and a month after the operation the man died, slowly suffocated. At the post-mortem examination Fig. 46. — Skeleton of an ossifying periosteal sarcoma of the femur. secondary deposits were found in the liver, pancreas, and ileum. The lungs were thickly occupied with secondary deposits ; and a large conglomerate mass as big as the fist compressed the trachea and adjacent segments of the bronchi. All the secondary deposits were hard and grated SARCOMAS OP BOKPS 81 under the knife, and some of tliem seemed to be con- tained in an imperfectly formed osseous capsule, or shell. In its general characters — the disposition of the Epiphysis. Epipliysial line. Cancellous tissue, with red marrow. Medullary canal. Periosteum. Sarcoma. BtROEAU Fig. 47. — Coronal section of the tibia of a girl with a periosteal sarcoma. She was alive and well five years after the amputation. secondary deposits — and in the mode by which it destroyed this man the sarcoma displayed thoroughly the usual features of an ossifying periosteal sarcoma of the femur. G 82 G0NNWTIVB-TI88UE TUM0UB8 This man had no notion that anything was wrong with his thigh until October, and by the middle of the following /3i-T7£/-y«|\TH-[c Fig. 48. —Tibia and fibula. The tibia is greatly expanded throughout its length by a central sarcoma. From a man 24 years of age. {Museum, Royal College of Surgeons.) 8JBC0MAS OF BONES 83 February he was suffocated by large secondary nodules of sarcoma compressing the bronchi. The tibia. — -Sarcomas are fairly common in this bone ; they prefer the upper to the lower end, and they do not Accessary nodule of sarcoma. Interosseous membrane. — Sarcoma. Flexor longus liallucis. Peroneus longus. — Detached portion of the flexor longus liallucis Fig. 49. —Spindle-celled sarcoma of the fibula. {Museum, Middlesex Hospital.) run such a rapid course as in the femur. For instance, I have had the opportunity of following nine cases of sarcoma of the fenmr throughout their whole clinical course. All the patients died within a year of operation from dissemi- nation of the tumour or from local recurrence. In the case of the tibia I have known several patients, who have 84 CONNECTIVE-TISSUE TUMOURS survived amputation of the leg for periosteal sarcoma, to be alive and in good health five years later (Fig. 47). A very large proportion of central tumours of the tibia, formerly classed as sarcomas, now rank with myelomas, and I am inclined to think that spindle-celled and round- Fig. 50. — Periosteal sarcoma of the upper portion of the fibula. The side figure shows the bone in section. celled central sarcomas of the tibia are rare tumours. The extraordinary manner in which a central sarcoma of the tibia will expand the bone is well shown in Fig. 48. The details of this remarkable case have been reported by Eve. The fibula. — This bone is not often attacked ; the upper end is the favourite situation, but periosteal sarco- mas may spring from any part of its shaft. (Figs. 49, 50, 51.) SARCOMAS OF BONES 85 Sarcomas of this bone are interesting because its upper half is vestigial, and its persistence is probably mainly due to the fact that it affords attachment to the muscles of the leg. The lower one-third has undergone ex- cessive development to meet the demands of the ankle-joint for greater security necessitated by the upright position in man. These facts induced me some years ago to depart from the usual rule in treating periosteal sarcoma of the fibula. We know that when these tumours at- tack the bones of the leg they do not run a very rapid course, so in a favourable case which came under my care in 1895 I resected the upper half of the fibula. The patient recovered with a very useful limb, and was able to walk about. Ke- currence took place in the scar eighteen months later ; this was removed. Six months after- wards a more extensive recur- rence rendered amputation a necessity. The patient died, two 3^ears and six months after the original operation, with signs indicating dissemination in the lungs. A careful examination of the literature relating to sarcoma of bone makes me think that these tumours are rare in the fibula, and certainly they do not run a very rapid course. The humerus. — Periosteal sarcomas of this bone are very dangerous to life ; they occur at all ages, and generally Fig. 51. — Fibula showiDg the change IDrocluced. by a central sarcoma growing in its upper end. 86 GONNEGTIVE-TISSUE TUMOURS involve the whole shaft o± the bone, and form large, soft, rapidly growing, spindle-shaped masses. Sarcomas situated at the upper end of the humerus have been very freely operated upon since 1887 by the interscapulo-thoracic method of amputation. The immediate results are good, but the remote consequences are dis- couraging. The radius and ulna. — Sarcomas of these bones, whether central or periosteal, are so rare that it is impossible to collect a sufficient number of cases to make deductions of any value. The few available records are sufficient to show that amputation has been followed by good consequences, immediate and remote. Some of these tumours, however, may have been myelomas. Clavicle. — Periosteal sarcomas of this bone are rare, and in nearly all the recorded cases have originated near the middle of the bone. A fair number of cases have been reported in which the bone and tumour have been suc- cessfully excised. Examples reported to be central sarcomas arose mainly in the sternal end, but these were in all prob- abiHty myelomas. Partial or complete extirpation of the clavicle does not impair the utility of the limb. Scapula. — It is easy to collect a score or more of records relating to sarcomas of the scapula. They arise mainly from the periosteum of the dorsal and ventral surface of this bone, and often assume formidable proportions. It is rare for sarcomas to arise in connexion with the pro- cesses of the scapula, but a central sarcoma of the coracoid process has been observed. Scapular sarcomas are usually of the spindle-celled species, and many of them chondrify and ossify, often very extensively (Fig. 52). Since 1887, when Berger introduced the operation known as interscapulo-thoracic amputation^ many surgeons have removed the scapula and upper limb in cases of scapular sarcoma. The immediate results of this formid- able operation are very gratifying, and though in a large proportion of the patients there is a quick recurrence, nevertheless life is more often prolonged than in amputation for sarcomas of many of the long bones. Occasionally, SARCOMAS OF BONES 87 when a sarcoma is confined to a limited area of the scapula, it is possible to excise the body of the bone, leaving the head in its normal relation to the shoulder-joint : some patients have recovered from this operation with a useful upper limb. Fig. 52. — Skeleton of a periosteal sarcoma of the scapula. {lluseum, St. Thomas's HQspital.) Innominate bone. — Sarcomas occasionally arise in con- nexion with this bone ; they may be periosteal or central, and may occur in any part of it. On the whole, the iliuni is the segment most commonly affected, and the tumours attain a great size. Stimulated by the success of the interscapulo-thoracic amputation for sarcoma of the scapula 88 CONNECTIVE-TISSUE TUMOURS attempts have been made to remove tlie innominate bone, or the greater part of it, with the lower limb, as a radical means of dealing with sarcoma of the ilium. This operation has been termed tbe interilio-abdominal amputation (Jaboulay, 1894). Keen and Da Costa have collected fifteen cases and added one under their own care. The results are not encouraging. Sternum. — This bone is sometimes the seat of primary sarcoma, and a few surgeons have excised portions of the bone with the hope of eradicating the disease. The results, immediate and remote, are not calculated to bring the operation into favour. Keen has reported a very suc- cessful example and collected the best-known cases. Ribs. — Sarcomas attack the ribs, and when they grow from the heads or necks of these bones are apt to send processes through the intervertebral foramina, which, extend- ing into the spinal canal, compress the cord. (Fig. 54.) A number of instances have been described in which surgeons have removed costal sarcomas, in some cases without opening the pleura ; but the results are not encouraging, and in the cases where the pleura was opened in the course of the operation the effects upon respiration and circulation were very grave. Webber, in removing a spindle-celled sarcoma of the sixth rib from a man 46 years of age, opened the left pleura and the peri- cardium. The jDatient recovered. Bones of the hand and foot. — Sarcomas of the meta- carpal and metatarsal bones, or the phalanges, are very ex- ceptional. Large, rapidly growing sarcomas arise from the tarsus, but it is unusual to find a central tumour in these cubical bones, though the}^ have been reported in the cal- caneum (Barthauer). Sufiicient facts are not available to enable anything like a satisfactory account to be furnished of the clinical course of sarcomas of the hands and feet; this is due to their rarity. Patella. — A sarcoma of this bone is a great rarity, but a careful report of a case has been published by Parker. The skull. — The large bones of the cranial vault — parietal, squamo-occipital, and the tabular portion of the SARCOMAS OF BONES 89 frontal — are liable to be attacked by periosteal sarcomas ; they grow rapidly, and form large tumours which cannot often be submitted to surgery. Pathological museums of any pretensions usually contain one or more crania exhibit- ing the peculiar formation of spiculated new bone charac- teristic of a periosteal sarcoma. The mesethmoid is an unusual situation for a sar- coma, but Moore has described an example which is interesting from the very extraordinary effects it pro- duced, for as the tumour increased in size it compressed the walls of the antrum and flattened out the body of each maxilla until these bones formed a thin expanded shell to the tumour, but the bones were not eroded or invaded by it. The sarcoma also greatly widened the space between the orbits and caused great deformity of the face, but did not invade the skull. There was no pain. Moore attempted the formidable task of removing this tumour, but the patient died during its progress. The parts are preserved in the museum of the Middlesex Hospital. Sarcomas arising in the muco-periosteum of the roof of the pharynx constitute an important clinical group under the name of naso-pharyngeal tumours. They are commonly met with in patients between the ages of 15 and 20, and in many cases arise from the muco-periosteum of the under-surface of the body of the sphenoid, and in some instances from that lining the sphenoidal sinuses. Such tumours sometimes extend into and plug one or both nasal fossse, processes of the tumour appearing at the nostril ; or they may extend downwards into the pharynx and impede deglutition. Sometimes the base of the skull is perforated by the tumour, and the patient dies of meningitis. Naso-pharyn- geal sarcomas often cause agonizing pain and intense frontal headache. Whilst the pain wears out the patient, his strength is further exhausted by frequently recurring and often profuse epistaxis. Exceptionally, a piece of the tumour will slough and become impacted in the larynx ; suffocation has followed this accident. Sarcomas of the jaws. — Although it is customary to speak of sarcomas which are connected with the maxilla 90 CONNECTIVE- TISSUE TUMOURS and mandible clinically as tumours of the jaws, it would be erroneous to describe them indiscriminately as tumours of bone. In each jaw there are, in addition to the bone and periosteum, two structures to consider — mucous membrane and teeth. In the case of the maxilla, the antrum requires to be considered, with its gland-containing muco-periosteum. Periosteal sarcomas of the jaws are rare before the fifteenth year, but they may occur at any age, even in Fig. 53. — Large recurrent sarcoma of the mandible. infants a few months old. They belong to the round- and spindle-celled species, and grow very rapidly (Fig. 53). These tumours are less frequent on the mandible than the maxilla ; they grow from any part of it Those which spring from the outer surface of the ramus are apt to be mistaken for parotid tumours. Periosteal sarcomas originate in any part of the maxilla, but they rarely arise from its facial surface, and, though fairly frequent on the gums, are very rare in con- nexion with the mucous membrane of the palatine process. The muco-periosteum of the antrum is a common situa- tion for these tumours, and as they grow they cause thin- SARCOMAS OF BONES 91 ning and expansion of the walls of tliis chamber. This enlargement of the body of the maxilla causes it to encroach on the nasal fossa and obstruct respiration ; often the tumour pushes up the orbital plate and displaces the eyeball (proptosis), and in a certain proportion of cases the alveolar border is depressed. The nasal duct is fre- quently implicated, and when it is completely obstructed epiphora is the consequence. Clinically, a sarcoma originat- Fig. 54. — Chondrifyiug sarcoma of the vertebrte and ribs. A portion of the tumour crept into the spinal canal and produced fatal paraplegia. {Museum, St. Bartholomew'' s Hospital.) ing within the antrum expands its walls, and by degrees processes of the tumour make their way through and implicate the skin of the cheek, or, projecting into the nasal fossa, ulcerate and give rise to frequently recurring hiEmorrhage. When the tumour perforates the posterior wall of the antrum, it will enter the zygomatic and spheno- maxillary fossfe, and creep thence into the temporal fossa, or make its way through the spheno-maxillary fissure and ramify in the orbit, or steal through the sphenoidal fissure or foramen rotundum into the middle fossa of the cranium. 92 CONNECTIVE-TISSUE TUMOURS Sarcomas of the palate. — The mucous membrane of the hard and soft palate is hable to mahgnant tumours be- longing to the sarcomas and squamous-celled carcinomas. It is also liable to a peculiar tumour which is somewhat rare, named " adenoma of the palate." These tumours are usually ovoid in shape, and vary in size from a cob-nut to a hen's egg ; they occur more frequently in the soft than in the hard palate, and are invariably encapsuled. These " palatine adenomas " are complex in structure. Some possess glandular tissue with ill-formed ducts and acini which in their structure mimic cancer, whilst the stroma in which they are embedded imitates sarcomatous tissue. They occur most strongly between the thirtieth and fiftieth years, but they have been met with at puberty. They are innocent tumours. They have been carefully studied by Stephen Paget and Hutchinson, jun. Many tumours described as sarcomas of the jaws are endotheliomas. Vertebrae. — Primary sarcomas of the vertebral column are rare tumours. They tend to invade the spinal canal and compress the cord (Fig. 54). It is very unusual for one to be amenable to surgical treatment, but Davies- Colley succeeded in removing one, and the patient, who was paraplegic, recovered motion and sensation. Secondary deposits of sarcoma and cancer occur with tolerable frequency in the spine ; and it is not an uncom- mon event for an individual to come under observation complaining of severe pain in the vertebral column, which may or may not be accompanied by a local swelling, proved by careful investigation to be due to a secondary deposit of malignant disease. In some of these cases the primary source of the disease was not known to exist until the " pain in the back " led to the examination. In one in- structive case mentioned by Horsley he actually operated on a spine for severe paraplegia, and discovered tumour- tissue in the arches and spine of the vertebra. Examina- tion determined it to be thyroid-gland tissue, and the patient had a goitre. There is one aspect of secondary malignant disease of the spine which needs consideration. When a deposit of sarcoma occupies bone, it softens the texture of the bone ; when this happens in the body of a SARCOMAS OF BONES 93 vertebra, especially of the lumbar set, the superincumbent weight will gradually compress and slowly efface the affected centrum. In some cases this is so complete that the intervertebral discs formerly separated by the diseased vertebra will come into apposition (Fig. 55). ^ . — Fig. 55. — A portion of the lumbar spine infiltrated with malignant disease slowly absorbed till intervertebral discs came into apposition. The pain which is set up by this slow " settling " of the column is very great, and may often be described as agonizing. I have noted its occurrence in the cervical as well as in the lumbar segments of the vertebral column. Barthauer, " Ueber die Extirpation des Calcaneus, nebst Beschreibung eines Falles von centralem Sarkom des Calcaneus, welcher durch Extir- pation des Calcaneus geheilt wurde." — Zeitschr. f. Chir., Bd. xxxviii. 462. Berger, "De 1' Amputation interscapulo-thoracique dans le Traitement des Jumeurs malignes de I'Extremite superieure de I'Humerus." — Reo. de CTJr., 1898, xviii. 861. Bland-Sutton, J., " On a Case in which the Upper Half of the Fibula was excised for a Sarcoma."— -Srft Med. Journ., 1896. i. 1086. 94 CONNECTIVE-TISSUE TUMOURS Davies-CoUey, N., " A Case of Fusiform Sarcoma of LaminEe of Dorsal VertebrEe : Pressure upon Spinal Cord; Eacbiotoray ; Cure." — Trails. Clin. Soc, 1892, xsv. 163. Eve, F. S., " Specimen of Central Fibro-Sarcoroa expanding Tibia, accom- panied by extreme Cystic Degeneration ; with remarks on the Relation of Injuiy and Inflammation to the production of Sarcoma of the Bones." — Trans. Path. Sue., 1S88, xxxix. 273. Hutchinson, J., jim., "Two Cases of Adenoma of the Palate, with ex- ceptional Clinical Features." — Trans. Path. Soc, 1886, xxxvii. 490. Keen, W. W., "Resection of the Sternum for Tumours, with report of two Cases and a table of seventeen previously reported Cases." — Med. and Surg. Meporter, 1897, Ixxvi. 385. Keen, W. W., and Da Costa, J. C, "A Case of Interilio-Abdominal Amputation for Sarcoma of the Ilium, and a synopsis of previously recorded Cases." — Internat. Clinics, vol. iv., 13th series. Moore, Chas. H., " Cranio-Facial Enchondroma." — Trans. Path. Soc, 1868, xix. 332. von Nasse, D., ' Die Geschwiilste der Speicheldriisen und verwandte Tumoren des Kropfes.",^ — Aroh. f. klin. Chir. (Langenbeck), 1892, xliv. 233. Paget, S., "Tumours of the Palate."— rr««5. Path. Soc, 1886, xxxviii. 348. Parker, Robert William, " Sequel to a Case • of Removal of Right Patella for Primary Sarcoma : Recurrence after six years in the Iliac Glands ; DQ&ih."— Trans. Clin. Soc, 1896, xxix. 22. Quenu et Longuet, "Tumours du Squelette thoracique." — Pev. de Chir., 1898, xviii. 365. von Volkmann, Rudolf, " Ueber endotheliale Geschwiilste, zugleich ein Beitrag zu den Speicheldriisen und Gaumentumoren." — Peutsche Zeitschr. f. Chir., 1895. xli. 1. Webber, H. W., "A Case of Sarcoma of the Sixth Rib in the removal of which the Pericardial and Left Pleural Cavities were opened; Vieco^Qxy."— Lancet, 1900, ii. 1347. CHAPTER VIII SARCOMAS OF GLANDULAR ORGANS Compound glandular organs like tlie kidney and prostate are liable to sarcomas at all ages, but these are more frequent Fig. 56. — Bladder and urethra in section : the prostate is occupied by a sarcoma. (From a hoy aged 7 years.) in the early years of life. This is well shown by sarcoma of the prostate : nearly half the recorded cases occurred in the first ten years of life, many of them during infancy. 95 96 GONNEOTIVE-TLSSUE TUMOURS Proust and Vian collected the records of thirty-four ex- amples of this disease ; the youngest was an infant of 5 months, the eldest a man of 73 years. Of these thirty- four patients, fifteen were under 8 years of age. As is the case with sarcoma generally, the onset and early course of the disease is very insidious and painless, until the tumour interferes with some important function. In the case of the prostate, it is the interference with micturition which draws attention to the existence of a tumour. In a boy under my own care the prostate was converted into a large mass which pushed the bladder high in the belly. The retention was caused by a bud-like process of the tumour which acted as a valve at the vesical orifice of the urethra. (Fig. 56.) The kidney is more frequently the seat of sarcoma than the prostate, but the disease shows the same relative frequency in early life. The type of sarcoma which grows in the kidneys of infants and children differs from that of adults. Renal sarcomas of infants. — These originate in the con- nective tissue of the renal sinus, and gradually distend the cortex until the tumour is surrounded by a thin capsule formed of expanded secreting tissue of the kidney. On this account these tumours are described as being encapsuled, but it is a spurious encapsulation formed partly by renal tissue and in part by the true capsule of the kidney. (Fig. 57.) On section, such sarcomas are yellowish-white, and the cut surface is often dotted with groups of small cavities due to secondary changes, especially when the tumour is very large. The base of such sarcomas is connective tissue con- taining cells of various shape and size ; some are round or oat-shaped, and others are spindles. In a fair proportion of specimens many of the spindle cells present the cross striation so characteristic of the fibres of voluntary muscle, and they lack a sarcolemma. When these cells are present the tumour is sometimes termed a myo-sarcoma. A careful microscopic study of these tumours, as well as a critical analysis of the descriptions published by others, indicates that when the striped cells are very SARCOMAS OF GLANDULAR ORGANS 97 abundant the tubules are, as a rule, absent. In examples containing many tubules (Fig. 58), as well as those in which striped spindles are numerous, the round, oat- shaped, and spindle sarcoma cells are equally abundant. It has been suggested by Paul that, as the most typical myo-sarcomas are more sharply delimited from the other varieties, the tubular elements may be derived from the kidney. I did not at first acquiesce in this view, but a more extended inquiry leads me to accept it. This is a Fig. 57. — Renal sarcoma in section ; removed from a child aged 20 months. matter worth consideration, because a study of the foetal kidney demonstrates very clearly that the renal sarcomas of infancy arise in the connective tissue of the renal sinus. The epithelial cylinders are due to the entanglement of uriniferous tubules, in consequence of the sarcoma invading the cortex, whilst the striated spindles are derived from the muscle-tissue of the renal pelvis, which is an expansion of the hollow muscle known as the ureter. These studies demonstrate in no uncertain way that renal sarcomas of infants are extrinsic in origin, and strictly non-renal. This view is now held by all who have carefully looked into the matter ; and it is worth mention that in 1857 van der Byl exhibited at the Patho- H 98 G0NNEGTLVE-TI8SUE TUMOURS logical Society, London, a large renal tumour, from a boy aged 8 years, which measured 82-5 cm. (33 inches) and weighed 31 pounds ; and in the description of the specimen in the catalogue of the Middlesex Hospital Museum, it is Fig. 58. — Microscopic characters of a renarsarcoma. (From an'infant of 20 months.) definitely stated that the growth appears to have sprung from the concavity of the kidney, and a narrow band of renal tissue can be traced round a great part of the circum- ference of the kidney. The general appearance of this boy in such dreadful circumstances is shown in Fig. 59. It is 8ABG0MAS OF GLANDULAR ORGANS 99 characteristic of these sarcomas that the ureter is rarely obstructed. This extraordinary freedom of the ureter from invasion explains the rarity of hsematuria in such cases, and, perhaps, what is otherwise remarkable, the painless- ness of these tumours in children, for there is no pressure ,vy^T«/c Fig. 59. — A boy aged 8 years with a renal sarcoma which weighed 31 pounds. from accumulated urine. A child with a very large renal sarcoma has been absolutely free from pain, and amusing himself with his playmates in the garden three days before he died. Indeed, many mothers, when the gravity of a renal tumour of this kind is explained to 100 GONNEGTIYE-TISBUE TUMOURS them, will express their astonishment that a child, apparently in excellent health and spirits, could be in such serious straits as the surgeon would have them believe. Though the ureter so constantl}^ escapes invasion, yet the veins are always implicated; and this constitutes one of the most peculiar as well as most dangerous features of renal sarcomas in children.' The tumour tissue extends into the renal vein, and often projects and even runs for a long distance into the inferior vena cava ; portions are detached and carried to the pulmonary circulation, and are arrested in the capillaries of the lung and originate secondary deposits. The intravenous apex of such an out- runner is usually cone-shaped and smooth. Occasionally a large fragment is detached, and this has been known to block the right auriculo-ventricular orifice (Osier). Such a gross embolus is uncommon. Plugging of the vena cava by an outrunner is by no means rare, and gives rise to oedema of the lower limbs. In a case under my own care the inferior vena cava was completely obstructed from its origin to its termination by a sarcomatous extension of this kind. It is a singular and well-established fact that when certain of the paired viscera, such as the kidneys, ovaries, eyeballs, and crura cerebri, are in early life attacked by sarcomas, in a very large proportion of cases, perhaps half the number, the disease is bilateral. In relation to this matter. Abbe recorded a very important observation. He successfully extirpated a kidney for sar- coma in a child 14 months old. Four and a half years later the little patient again came under his care with a sarcoma in the remaining kidney. In 1893 I collected and tabulated in the first edition of this book twenty-one complete records of renal sarcoma in infancy which had been submitted to nephrectomy. In the list of twenty-one cases, twelve patients died as a result of the operation ; of those which recovered, all died of recurrence within a year. Since the publication of that table a large amount of interest has been aroused in the question of the results of nephrectomy for sarcoma, and it is now an easy matter to collect a hundred records. The analysis of a SAUGOMAS OF GLANDULAR ORGANS 101 large number of these reports sliows that nephrectomy for renal sarcomas in children under 6 years of age has a mortality of over 50 per cent. Of the fifty that recover, forty-five die from recurrence at periods varying from two months to a year. In the remaining five, life may be prolonged, as shown in the adjoining table : — • RENAL SARCOMAS IN INFANTS Table of cases in which life mas prolan r/ecl beyond one year by nephrectomy Reporter Age Result Hicquet . . Schmidt . . Abbe .... Abbe .... Malcolm . . 6 months 6 months 2 years 1 yr. 2 mths. If years Died li years after operation. {Acad. Roy. de Med.^de Belgique, Jan. 28, 1882.) Alive and well thre • years later. (Dr. Emily Lewi, Arch, of Pediatrics, xiri. 97.) Alive and well five years later. {Ann. of Sury., 189i.) Patient died 4 J years later from sarcoma in re- maining kidney. {Ann. if Surg., 1894 and 1897.) Alive and well 10 years later. {Trans. Clin. Soc, xxvii. 94, and private letter.) It is very certain that a child with a renal sarcoma runs an enormous risk of losing its life when submitted to nephrectomy, and at the same time the chances of pro- longing life are more slender than in any other surgical operation. It must, however, be borne in mind that the disease is surely fatal within a very limited period when allowed to run its own course. Renal sarcomas of adults. — These differ in many impor- tant particulars from the sarcomas of infancy. In the first place, a sarcoma in the adult arises in the cortex, usually in connexion with the capside, and then gradually invades the true tissue of the kidney. The relation of renal sar- comas to the capsule is of some importance, because similar tumours arise in the connective tissue in which the kidney is embedded ; these are perirenal sarcomas, and, as far as my observations go, this is a more frequent position for them than those which we term renal sarcomas. A careful comparison of these tumours leads me to believe that, in the adult, sarcomas of the type represented in 102 OONJ^EGTIVE-ttSSU:^ TUMOtM Fig. 60 have their origin in the renal capsule, whereas the sarcoma of childhood arises, as already pointed out, in the connective tissue of the renal sinus. This is a subject of some interest, because a critical comparison of the mode of origin of sarcomas in viscera similar to the kidney, e.g. the spleen, thyroid gland, and prostate, shows that such tumours are not only uncommon, but are often closely connected with the connective-tissue investments of such organs. Fig. 60. — A kidney in section with a sarcoma invading its cortex. From a man 51 years of age. {Museum, 3fiddlesex Hospital.) Treatment. — The only available treatment for renal sar- coma is early excision of the affected organ. This is rarely of much service. The mortality of the operation is now very small, but recurrence usually takes place within a year. A new interest was given to malignant tumours of the kidney when Grawitz stated that many of them occurring in adults exhibited the structure of the zona fasciculata of the adrenal. Grawitz further suggested that these tumours, now known as hypernephromas, probably arose in detached (ectopic) pieces of the adrenals (accessary adrenals). It has long been known that accessary adrenals are found SARCOMAS OF GLANDULAR ORGANS 103 beneath the capsule of the kidney (Fig. 61), as well as on the under surface of the liver ; they are also found in the retro- peritoneal tissue in the course of the spermatic artery, in the spermatic cord, simulating fatty tumours (Andrewes). In addition to the topographical and histologic features of these tumours, stress is laid on the presence of glycoo-en in the cells, this being an additional support to the view that such tumours arise in embryonic tissue. ACCESSARY , DRE /^ Fig. 61. — Au accessary adrenal beneath the capsule of the kidney. {Maseum, Royal College of Surc/eonti.) It has long been known that the adrenal may be trans- formed into a large tumour in the same way that the thyroid gland becomes a goitre, and the analogy is so striking that Yirchow years ago proposed for adrenal tumours the term "struma suprarenalis." In recent years this analogy has been further justified by the fact that some of these adrenal tumours, as well as those which arise beneath the capsule of the kidney, and exhibit the adrenal structure, disseminate and mimic the extraordinary phenomenon 104 GONNEOTiVE-TISSUE TUMOTJUS known as " general thyroidal malignancy," in which tumours, exhibiting all the microscopic features of thyroid gland, appear in the bones, especially the skull, vertebrae, and femur, as well as in the viscera, in association with what appears to be a simple adenoma of the thyroid gland. Whatever view may be taken of the tissue in which these tumours arise, it is quite certain that they exhibit peculiarities of structure which distinguish them from the Fig. 62. — Sarcoma of the kiduey supposed to arise from an adrenal "rest." Removed from a woman aged 42 years, during pregnancy. She was in good health five years later, in spite of having borne a child. ordinary round- and spindle-celled species of sarcomas. That they are malignant is equally beyond question, for they recur after removal and give rise to secondary nodules in the lungs. The frequency with which the lungs are infected is due to the primary tumour invading the renal vein. Though these tumours are very vascular, and their central parts often destroyed by extravasation of blood, they do not give rise to hsematuria because the tumour does SAUGOMAS OF GLANDULAR ORGANS 105 not invade the renal pelvis. This is the most striking fact in their clinical history. Recently doubt has been thrown on the origin of hyper- nephromas from islands of adrenal tissue, especially by Stoerk. He points out that these tumours do not occur in the adrenal itself; also that adrenal rests are most frequently found in the upper pole of the kidney and hypernephromas occur more commonly in the lower half of this organ. Adrenal rests occur in the liver, but hypernephromas have not been detected in hepatic tissue. The opinion is gaining ground that the majority of tumours classed as hypernephromas are carcinomas arising in the cells of the renal tubules, and I share in this opinion. The classification of malignant renal tumours is a difficult matter. van der Byl, " Large Cancerous Growth of the Kidney in a Child." — Trans. Path. Soc, 1856, vii. 268. Edington, G. H., Myxo-Sarcoma of the Prostate in a Child aged 1 year and U months— Brit. Med. Journ., IS09, ii. 754. Trotter, W., " On Hypernephroma." — Lancet, 1909, i. 1581. CHAPTER IX TUMOURS OF THE ADRENAL (SUPRARENAL CAPSULE) The adrenal is liable to tumours, many of which, have been described as sarcomas, some as carcinomas, and others as hypernephromas. The adrenals, like other paired organs, are subject to malignant tumours at two distinct periods : childhood and adult life. Adrenal tumours in children. — Our first knowledge ot these tumours, in the main described as sarcomas, was derived from post - mortem observation. This evidence showed that such tumours were rare, that they occurred in the early years of life, usually attacked both organs, and sometimes attained the size of coco-nuts. It was also established that they gave rise to secondary deposits, especially in the liver. Observers like Greenhow, Hale White, Dalton, Ogle, Dickinson, Colcott Fox and others not only gave careful descriptions of»the tumours, but some of them drew attention to the peculiar coloration of the skin, unlike the bronzing of Addison's disease, the abnormal development of hair, and in some instances precocious development of the sexual organs. Many carefully described examples have since been published. Bulloch and Sequeira have collected twelve cases in which the ages of the patients varied from 1 to 14 years. The majority of the children were girls under 4 years. This combination of pigmentation, precocious develop- ment of the sexual organs, and a tumour of the adrenal is so remarkable that it is necessary to give brief details of two well-marked examples. Dr. Sequeira's patient, aged 11 years, looked like a stout little woman of 40. She was four and a half feet 106 TUMOmS OF THE ADRENAL 1('7 high and weighed eighty-seven pounds, a brunette, with coarse skin, and a copious development of hair on the lips and chin. The pubic region and axillae were covered with long hair, and her mammse resembled those of a sexually mature woman. The abdomen was distended with fluid (hydroperitoneum), and a large tumour could be felt in the left hypochondrium. She died a few months after coming under observation. The left adrenal was replaced by a tumour weighing three pounds. The liver and lungs contained secondary deposits. The microscopic structure of the tumour and the secondary deposits resembled that of the cortical portion of the adrenal. This girl up to the age of 10 years had been to all outward appearance normal. A case recorded by Adams is equally remarkable. The patient, a boy aged 14 years, developed normally to the tenth year, then he became pubic, this change being accompanied by marked muscular development, and the growth of a beard so abundant that he had to be shaved almost daily. His appearance was that of a sturdy little man. His complexion grew dusky, and a tumour became obvious in his abdomen. An attempt was made to remove the tumour, but it proved inoperable : the boy died eighteen months later. The tumour weighed eight and a half pounds and adhered to the left kidney. No trace of the left adrenal could be found. The liver was thickly dotted with secondary deposits, some of which were as big as walnuts. Microscopi- cally the tumour presented an alveolar arrangement, and was regarded as a hypernephroma, taking its origin in the cortex of the left adrenal. These important observations indicate that the cortex of the adrenal is probably connected in some way with the growth of the body, and the development of puberty and sexual maturity. Guthrie and Emery, following up these observations, have pointed out that precocious obesity is sometimes associated with hypernephromas and forms a clinical feature as striking as precocious puberty. Parkes Weber considers that the extraordinary development of children associated with the presence of a h3^pernephroma presents two types, (1) the precociously obese type (Fig. 63), and (2) the muscular or " infant Hercules " type. 108 CONNEGTIVE-TISSUE TUMOUliS From a careful consideration of the subject, Guthrie and Emery come to the conckision that precocious physical de- velopment, sexual and somatic, may be due to tumours or Fig. 63. — A toy 4| years of age, the subject of precocious obesity associated with a hypernephroma and acute tuberculosis. He resembles in miniature "a burly brewer's drayman" {Guthrie). TUMOURS OF THE ADRENAL 109 hypertrophy of the pituitary and pineal glands, and of the adrenal cortex. Premature hirsuties occurs in practically all cases of premature physical development, but is not necessarily associated with other signs of sexual maturity. The obese type of precocious development may occur in boys and girls, but the muscular type is confined to boys. It is necessary to remember that precocious development, sexual and somatic, may be unassociated with any obvious lesion of glandular organs. Adrenal tumours in adults. — Malignant tumours arise in the adrenals of adult men and women : they sometimes attack both organs, and display the usual features of malignancy, for they grow rapidly, disseminate, and quickly destroy life. Adrenal tumours in adults, as in children, are sometimes associated with unusual hairiness (hirsuties). Thornton recorded a case of this kind. He removed from a lady 3G years of age a large tumour of the left adrenal. The patient was covered " with long, silky hair, and had to shave her face just like a hairy man." The tumour was removed in April 1889, and in November of the same year she wrote that she was like her old self and had " all the external appearances of other women." The tumour removed from this patient is preserved in the museum of the Royal College of Surgeons of England. Adams, C. E., "A Case of Precocious Development associated with a Tumour of the Suprarenal Body."— Travis. PatJi. Soe., 1905, Ivi. 208 ^ Bulloch, W., and Sequeira, H., "On the Relations of the Suprarenal Capsules to the Sexual Organs." — Trans. Path. Soc, 1905, Ivi. 189. Dalton, N., " Infiltrating Growth in Liver and Suprarenal Capsule." — Trans. Path. Snc, 1885, xxxvi. 247. Greenhow, E. H., " Cancer of One Suprarenal Capsule." — Trans. Path. Soo., 1867, xviii. 260. Guthrie, L., and Emery, W. d'Este, " Precocious Obesity, Premature Sexual and Physical Development and Hirsuties in relation to Hypernephroma and other Morbid Conditions." — Trans. Clin. Soc, 1907, xl. 175. Ogle, John W., " Unusually Large Mass of Carcino.natous Deposit in one of the Suprarenal Capsules of a Child." — Trans. Path. Soc, 1865, xvi. 250. Thornton, J. Knowsley, " Abdominal Nephrectomy for Large Sarcoma of the Left Suprarenal Capsule: Recovery." — Trans. Clin. Soc, 1890, xxiii. 150. West, Samuel, " Primary Sarcoma of the Suprarenal Capsule, with Secondary Growth in the Lung."— Trans. Path. Soc, 1879, xxx 419. White, W. Hale, " Sarcoma of Suprarenal Capsule " — Trans. Path. Soc, 1885, xxxvi. 464. CHAPTER X PIGMENTED TUMOURS This chapter will be devoted to tlie consideration of melanomas and pigmented conditions such as the small red plaques on the skin known as " De Morgan spots," ochronosis, chloroma (green cancer), and xanthoma. MELANOSIS In the majority of mammals there are certain epithelial and connective tissues which normally contain pigment. Among pigmented tissues the skin and the epithelial layer of the retina hold the first place. In skin the pigment is chiefly contained in the deeper layers of the rete mucosum ; and hair, being derived from the cells of this layer, is pigmented also. In many mammals other tissues contain pigment, such as the mucous membrane of the roof of the mouth of the dog, and the blue coloration of the vaginal mucous membrane of the vervet monkey. In man the amount of pigment varies greatly, so that we may pass gradually from individuals whose skins are intensely black to others who have no trace of cutaneous pigment. It is a noteworthy fact that animals with no pigment in the skin also lack pigment in the uveal tract of the eyeball. A familiar example of this is the white rabbit with pink eyes. Such a condition is termed albinism, and colourless animals, or albinos, occur among all classes of animals, vertebrate and invertebrate. Excessive development of black pigment in the skin is known as melanism ; this is much rarer than albinism. Abnormal distribution of pigment is common ; in man it gives rise to the condition termed leucoderma when it affects the skin, and unequal distribution of pigment in the retina is 110 MELANOSIS 111 known as retinitis pigmentosa. Irregular patches of black in the skins of horses cause them to be described as piebald, and when disseminated in small dots and irregular tracts they are said to be grey. In the white races of men the pigment granules are almost entirely confined to the cells of the rete mucosum, but when the pigmentation is very marked it will be found distributed in the other tissues of the skin. The pigment, or melanin as it is called, lies within the cells in the form either of black or of brown granules, or they may be uniformly Fig. 64. — Anterior portion of a dace ; each black spot contains a central white dot representing au encysted parasite. stained by it. It is stated that white skin transplanted on to a negro soon becomes pigmented, and that when the skin of a negro is grafted on to a white man it undergoes depig- mentation. It has long been known that leucocytes carry pigment. In amphibians and fishes pigment occurs in the branch- ing cells (Deiter's cells) situated beneath the epidermis. These cells are filled with black melanin granules, obscuring the nucleus. On exposure to light these protoplasmic processes retract, and the pigment is concentrated in the cell body, but when kept in the dark the processes are protruded and the pigment is diffused in the surrounding structures. The most remarkable example of pigment-formation is found in cuttle-fishes (octopus and sepia). These inverte- 112 G0NNEGTIVE-TI88UE TUMOURS brates possess an ink-bag from whicb, when irritated, they eject a black pigment (sepia) in such abundance as to colour the surrounding water to the extent of a cubic yard or more, and under cover of this dark cloud they escape from their enemies. Melanosis is sometimes produced by parasites. This variety of melanism is rarely seen in man, but is fairly frequent in dogs and fishes (Fig. 64). Pathological pigmentation in its most serious form is seen in connexion with tumours known as melanomas. Melanomas. — Melanomas are rare tumours, which occur in those regions of the body where pigment is found normally, namely, the skin and the pigmented tissues of the eye. Melanomas of the skin arise in abnormal pigmented areas such as moles and warts, and especially in patches of pig- ment sometimes found near the matrix of the nails on the fingers and toes, and occasionally they follow injuries of the skin. We have been accustomed to classify these malignant tumours roughly as round-celled or spindle-celled sarcomas according to the prevailing type of cell visible in magnified sections ; but there is another, but very rare, kind detected by Collins, which arises from the glandular epithelial cells in the ciliary body. Ribbert has published some observations (1897) which add great interest to these tumours. A¥hen the choroidal tumours are examined by teasing, the cells assume the stellate forms Avith which most of us are familiar in the pigment cells so abundant in the skin of frogs and other amphibians, and particularly in cuttle-fishes. These are known as chromatophores, and Ribbert would have us so regard' these cells in the choroid and in the skin, and he even suggests that melanomas might be appropriately termed chromatophoromas. In regard to this, it is necessary to point out that the black pigment (melanin) in the pigment cells of vertebrates only agrees (according to MacMunn) with that of invertebrates in the common attribute — blacknes;! The interest of Ribbert's observations lies in the fact that in secondary nodules of choroidal tumours found in the liver and brain, the cells were identical with the chromato- MELANOMAS 113 phores which are so characteristic of the lamina fusca element of the choroid. The amount of melanin present in pigmented tumours varies greatly ; in some it is so small that the tissue takes on merely a brown coloration, in others the tissues are as black as ink. The pigment particles are lodged in and among the cells of the tumours, even in the walls of Fig. 65. — Pigmented stellate cells from a melano -sarcoma. The cells in the lower part of the figure are from a secondary nodule in the liver ; the upper from a metastatic nodule in tlie brain. The primary tumour iu each instance arose in the choroid. Teased specimens {after Ribbcrt). the blood-vessels. Occasionally the secondary nodules are blacker than the primary tumour. Melanin. — This occurs as fine, irregular, amorphous granules varying from light brown to intense black. It is soluble in ether, alkalies, and strong acids, and is bleached by \ 114 CONNECTIVE-TISSUE TUMOURS chlorine — a fact which is useful in examining the microscopic features of melanomas. The urine of patients with melanotic tumours often contains black pigment (melanogen), usually in solution, but occasionally suspended in the form of granules. The urine is as a rule clear when first voided, but blackens on exposure to the air, and becomes intensely black when submitted to oxidizing reagents, e.g. a mixture of sulphuric and hydro- chloric acids to Avhich a few drops of ferric chloride have been added. A more sensitive test is the addition of bromine water to the urine, wdiich yields a yellow precipitate turning black on exposure to light. I made several observations on the urine of patients suffering from melanomas, in the hope that some opinion might be formed as to the gravity of the patient's condition according to the amount of melanin present. I am inclined to believe that an abundance of melanin is of the gravest import. In the rare anomaly known as alkaptonuria the urine when passed is clear, then becomes brown, and finally black on exposure to the air. {See Ochronosis, p. 120.) Primary melanomas of the skin. — These arise in moles, especially the black blemish known as navus spilosus, and in pigmented warts. A pigmented mole may remain quiescent throughout a very long life and never cause the least inconvenience ; in other instances, fortunately rare, as life advances the mole ulcerates, perhaps bleeds freely, and may even partially heal ; but coincidently with the onset of ulceration the adjacent lymph-glands enlarge, become charged with pigment and sarcomatous tissue, spaces filled with inky fluid' form in them, and finally the overlying skin ulcerates. The infection may not proceed farther chan this ; recurrent liaBmorrhage from the fungating glands, or furious bleeding should a large vein or artery become broached by ulceration, carries off the patient. In many cases the morbid material is transported into distant parts, secondary knots form in the liver, lung, kidney, or brain, and death arises from interference with the functions of these organs. In other cases the mole, instead of ulcerating, is observed MELANOMAS 115 to become more prominent, and finally forms a tumour of some size standing out prominently from the skin. In due course the lymph-glands in anatomical relation with the part from which the tumour arose enlarge, and secondary deposits occur in the viscera, bones, or skin. It does not necessarily follow that in all cases of melano- mas arising in moles secondary deposits are formed in the viscera. In some cases — which, however, are very rare — the tumour seems to become mainly a source of pigment, largo quantities of which enter the circulation, to be discharged with the urine. Exceptionally the skin assumes a dusky tint. Many melanomas arise in pigmented warts, especially the solitary congenital kind. After middle life such a wart may grow, ulcerate, stink, cause the adjacent lymph-nodes to enlarge, and then infect the system with secondary nodules. Melanosis in connexion with the fingers and toes assumes two forms : it may occur as a deep pigmentation of the skin, usually in the immediate neighbourhood of the nail, often involving the matrix, and even the nail itself; or a small pigmented nodule will arise in the nail matrix or in the adjacent skin, and ulcerate, dissemination following. The hallux is the digit most prone to be attacked by a melanoma, and several examples have been carefully recorded, most of the patients being women. The cases are arranged in the following table : — Reporter Sex Age Digit Referexce Fergusson . Hutchinson Nunn . . Lediard . . Bowlby . . Barnard . . M. F. F. F. F. F. 36 60 50 40 55 60 Hallux . . Hallux . . Fifth finger Index finger Hallux . . Index finger Lancet, 1857, i. 290. Trans. Path. Soc, viii. 404. Ibid., xxxi. 299. Ibid., xxxix. 307. Ibid., xli. 314. Brit. Med. Joiirn., 1902, i. 457. Apart from abnormal deposits of pigment such as moles and warts, melanomas are occasionally found in those parts of the body where the skin is more deej^ly pigmented than usual, namely, the external genitals in both sexes, and the skin around the anus. The skin of the vulva, male genitals, and the anus contains more pigment than other parts of the body. Malignant 116 CONNECTIVE-TISSUE TUMOURS melanoma of the vulva is rare. Holland collected 37 cases. Melanoma of the penis is very rare. Cases have been reported by Fischer and Payr. Primary melanoma of mucous membrane is very rare, and it is odd that the recorded cases have been observed on the muco-periosteum of the hard palate. Treves, in recording an example, reminds us of the fact that the mucous membrane in this situation is charged with pigment in certain mammals. This is the case Avith dogs. The theories relating to the connexion between abnormal patches of pigment, moles, and melanomas have recently been carefully summarised by Fox. Intra-ocular melanomas. — The commonest situations of these tumours are the uveal tract and the lamina fusca; the seat of origin accounts for the presence of pigment. Their distribution in these tissues is curious, for a melanoma of the choroid is ten times more common than in the ciliary body, and a pigmented tumour of the iris is excessively rare; moreover, those Avhich arise in the ciliary body are carci- nomas. Melanomas of the uveal tract are most frequent between the fortieth and sixtieth years, but they have been observed as early as the fifteenth and as late as the eighty-fourth year. In structure they may be round-celled, spindle-celled, or mixed-celled, the size of the cells varying greatly in different tumours. The amount of pigment, too, in intra-ocular melano- mas varies greatly ; in some specimens it is so abundant that the tumour is coal-black ; in others it is only sufficient to impart a grey tint. Occasionally the pigment is so irregularly distributed that some parts of it are almost colourless. The tumour remains for a time restricted to the interior of the globe, but it tends to escape therefrom in three directions : («) along the course of the venoe vorticosfe, appearing outside the sclerotic in the situations where these veins emerge ; (6) the presence of the tumour leads to an increase in the intra-ocular tension, and finally to sloughing of the cornea ; {c) the growth may invade the optic nerve. Melanomas are very apt to recur after removal, and to become disseminated. The most frequent situation in which INTBA-OGULAR MELANOMAS 117 to find secondary deposits is the liver; but any organ may contain them, even the bones. It is surprising, con- sidering that the eyeball is near to and in such close relation with the brain by so large a nerve- trunk as the optic nerve, that the brain should be rarely implicated. It is a fact that Avhen the brain is a seat of deposit this is rarely the result of extension along the nerve. The amount of dissemination varies greatly: in some cases secondary knots occur in almost every organ ; in others they will be limited to the liver. The lymph-glands adjacent to the orbit are rarely infected. It is curious that in most cases death results more often from the secondary growths involving important organs than from the local effects of the primary tumour. A rare complication of melanotic tumours is pigmentation of the skin. The duration of life in patients with intra-ocular melano- mas rarely extends beyond three years. A careful analysis of a large number of cases shows, however, that in many instances life may be indefinitely prolonged by early removal of the globe, and cases are known in which patients have been reported alive and well five, six, eight, nine, sixteen and eighteen years after the operation. In the majority of cases that recur the recurrence takes place within three years of the operation. Collins and Lawford, calculating cases in which recurrence does not take place within three years of operation as recoveries, come to the conclusion, from an analysis of seventy-nine cases of which they were able to obtain complete records, that the rate of recovery is 25 per cent., but they point out that patients have died from recurrence or secondary deposits after a much longer interval than three years. Dissemination has been deferred for so long a period as eleven years after excision of the eyeball (Hutchinson). Melano-carcinomas. — Several writers, who have devoted attention to intra-ocular tumours, describe some of the pigmented tumours as carcinomas, using the term in the definite sense in which it is employed in this work. Much new light has been thrown on this question by the interesting investigations of Treacher Collins. This ophthalmologist has demonstrated the existence in the ciliary body, in the space 118 GONNEGTIVE-TISSUJS TVMOtJRS extending from the root of the iris to the ora serrata, of a number of small tubular processes composed of epithelial cells with the free ends projectinf^ towards the ciliary muscle (Fig. 66). Collins succeeded in demonstrating the existence of these processes by bleaching the cells. The ciliary glands are interesting in connexion with melano-carcinoma, for Collins discovered among the intra- ocular tumours preserved in the museum of the Moorfields Hospital two examples from the ciliary body which were epithelial in character. In examining them he adopted Fig. 66.— Bleached section of the glands of the ciliary body. {After Collins.) the bleaching method, to which reference has already been made. We must agree with his observation, that melano- carcinoma is unknown except as a primary tumour arising in the ciliary body. Melanomas are of fairly common occurrence in horses : the regions most affected are the tail and the parts about the anus, where they form large mushroom-like excres- cences, with little disposition to ulcerate. The tumours in some cases attain large proportions, and have been known to weigh forty, fifty, and even sixty pounds. When a large tnmour grows from a horse's tail it becomes a great encum- brance, which the veterinarian removes by amputation. It GANGEBODEBMS 119 occasionally happens that in the operation a portion of the tumour is left behind, and its cut surface heals like other tissues. These pigmented tumours are very prone to dis- seminate, and secondary nodules occur in almost all the viscera ; in spite of this, melano-sarcoma does not appear to be such a malignant affection in horses as in men. Although most common in grey, it also occurs in white, and occasionally in black horses ; and it certainly occurs in cows. Next to the anus and tail, the udder is the most frequent seat of the primary tumour, and it may spring up in the subcutaneous connective tissue in any part of the trunk. Horses may be attacked at any age from four years upwards. In structure, melano-sarcoma of the horse resembles a hard uterine fibroid rather than a sarcoma. In these aninials melano-sarcoma of the uveal tract is very rare. Sarcoma idiopathicum multiplex hsemorrhagicum. — ■ This rare disease, described by Kaposi, has been most fre- quently seen in Polish Jews. It attacks the feet and hands and gradually extends up the limbs. The skin involved in this disease in the most typical cases is bluish-red, and the nodules, which tend to become confluent, vary in size from a split pea to a hazel-nut. The tumour-nodules are very vascular, and on microscopic examination resemble spindle- celled sarcomas. The coloration is due to blood, so that this disease is quite different from melanoma. De Morgan spots (canceroderms). — ^It is not uncommon to find on the skin, especially of the abdomen and chest of patients debilitated by cancer, numbers of small raised red spots looking like na3vi. These are often called " De Morgan spots," after Campbell De Morgan (1872), who regarded them as almost pathognomonic of cancer: they are patches of pigment, and not nsevi. These spots have been carefully studied by Brand and Leser. Their conclusions are of interest, for they point out that the J spots do not appear in healthy subjects, or in persons suffering from other diseases in early or middle life, and never even in old age in large numbers. When these spots are plentiful there is every reason to suspect cancer. I have made careful observations of them for twenty-five years in regard to their association with cancer, and find 120 CONNECTIVE-TISSUE TUMOURS that they are as common in the non- cancerous as in those afflicted with this disease. Ochronosis and alkaptonuria. — For many years after Tirchow, in 1866, drew attention to the peculiar discolora- tion of the cartilaginous tissues of the body under the designation ochronosis, it may be said to have remained a pathologic curiosity, until Albrecht (1902) drew attention to the occasional relationship which exists between this disease and the curious and rare condition of the urine known as alkaptonuria. The condition termed ochronosis scarcely amounts to a disease, as it in no way shortens life, and in the early cases the changes, which consist of blackening of the costal cartilages, the gristly parts of the pinna and sclerotic, were only discovered at a post-mortem examination. In cases sub- sequently reported, such fibrous structures as the inter- vertebral discs and the chordae tendineee have been found discoloured, and in a remarkable case, recorded by Pope, the rib cartilages were blue-black, the ears w^ere blue, there were black patches on the inside of tho ^lips, and the skin of the face had broAvn patches not unlike the pigmentation of Addison's disease. On microscopic examination of a patch of pigmented skin from a patient with ochronosis, the pigment particles were found in the elastic tissue of the skin, but not in the rete Malpighii. Osier has reported a case in which there was skin pigmentation. Alkaptonuria has been particularly investigated by Garrod; its essential features are as follows: The urine, though of normal appearance when passed, acquires a deep brown colour and ultimately becomes black on exposure to the air. The brown colour is intensified by alkalies. The urine reduces Fehling's solution with the aid of heat, and actively reduces ammoniacal silver nitrate solution in the cold. Fabrics moistened with alkaptonuric urine became deeply stained on exposure to the air. This anomaly often dates from infancy, and, in one case at least, staining of the napkins by the urine was noticed the day after birth, Garrod, m his classical analysis of this disorder, states that " homogentisinic acid is a constant constituent of XANTHOMA l2l alkapton urines, and plays the chief part in the production of alkaptonuria." In regard to the relationship between ochronosis and alkaptonuria, Garrod writes : " There are very strong grounds for believing that in later life alkaptonuric subjects tend to develop the characteristic pigmentation of cartilages ; in other words, that alkaptonuria is a cause, but not the only cause of ochronosis." Chloroma (green tumours). — This is an exceedingly rare disease in which sarcoma-like masses form on the bones of the skull and face, especially in the neighbourhood of the orbits, and infect other organs secondarily. After death the colour of the tumour-like masses is grass-green. The nature of the disease is obscure : some writers regard it as a form of leukaemia. It has been carefully studied by Melville Dunlop. Xanthoma. — This, with its many synonyms, is a curious, harmless pigment disease, especially liable to appear in the skin of the eyelids near the inner canthus. Histologic- ally it consists of a fibrous and fatty tissue containing yellow pigment and connected with the corium. In the eyelids the disease is usually symmetrical and occasionally congenital. These early cases have led some observers to regard the disease as allied to nesvi. Xanthoma may occur on any part of the skin (A''. viultiplex), and in many instances is associated with jaundice and disturbance of the liver. The orange-coloured pigment is interesting from a phy- siological point of view in connexion with the oil-gland of the rhinoceros hornbill (Bucorvus abyssinicus). In this bird the gland secretes an orange-coloured material with which it preens its feathers. The only normally pigmented tissue found in the human body resembling the yellow and orange of xanthoma patches is the lutein tissue in the corpus luteum and the Avails of lutein cysts arising therefrom. Brand, " Canceroderms."— ^/-i^. Med. Journ., 1902, ii. 494, 730. Collins, E. Treacher, " Cysts of the Glands of the Ciliary Body. Researches on the Anatomy and Physiology of the Eye," London, 1900. Collins and Lawford, " Notes on Three Hundred Cases of Sarcoma of the Uveal Tract." — Roy. Lond. Ojjhthal. Hosp. Repts., 1891, xiii. 104. 122 CONNECTIVE-TISSUE TUMOURS Dunlop, " Chloroma." — Brit. Med. Journ., 1902, i. 453. Fischer, G., " Melanosarkom der Penis," — BeuUclie Zeitschr. f. Cldr., 1887, XXV. 313. Fox, Wilfred S-, " Researches into the Origin and Structure of Moles, and their Relation to Malignancy." — Brit. Journ. of Berm., Jan. 1906. Garrod, "A Contribution to the Study of Alkaptonuria." — Med.-Chir. Trans., 1899, Ixxxii. 867. Holland, E., " Malignant Melanoma of the Vulva." — Journ. of Oistet. and Gyn., 1908, xiv. 809. Hutchinson, J., jun., '• Melanotic Sarcoma of the Skin." — Trans. Path. Soc, 1898,xliv. 148. Kaposi, " Sarcoma idiopathicum multiplex hfemorrhagicum." — Bandatlas der HautliranTilieitcn, Leipzig, 1900, Tafeln 301, 302. Payr, E., " Melanom der 'Penis.'"— Be^itscJie'Zeitschr.f. Chir., 1899, Bd. liii. 221, Pope and Gaxrod, " A Case of Ochronosis, with table of eleven cases previously reported." — Lancet, 1906, i. 24. Treves, Sir Frederick, "Melanoma of the Hard Palate." — Trans. Path. Soc. Land., xxxviii, 350, CHAPTER Xr MOLES Moles are pigmented and, usually, hairy patches of skin. These patches are congenital, and vary greatly in size ; many are no bigger than split peas, others cover an extensive area on the face, trunk, or limbs. The common variety consists of a slightly raised brown patch ; it is sometimes quite black, and is, as a rule, covered abundantly with hair, which is commonly short (ncevus pilosus) ; occasionally it is as long as that naturally found upon the scalp. The hairs are furnished with sebaceous glands, and sweat-glands are often present. The amount of pigment varies ; occasionally it is so abundant as to produce an inky blackness. Some black blemishes are glabrous (ncevus spilosus). Moles are very vascular, but the most striking feature of their histology is the fact that the tissue immediately underlying them is arranged in alveoli. The most important change to which they are liable is to become later in life the starting-point of melanomas, some of which are very infective, and quickly destroy life. When very large moles occur on the trunk the hairy part is sometimes very sensitive, almost hypersesthetic. In large moles, pendulous skin-folds are sometimes present ; these folds are large in the young, but, as a rule, they shrink and become quite small in the adult. As many as fifty moles may be present on one individual. When a mole is extensive, and occurs in an exposed situation, it is a serious disfigure- ment. The relation of moles and plexiform neuromas is described on p. 140. Small hairy moles do not, as a rule, cause much in- convenience even when they occur on the face, in which situation they are known as " beauty spots." A small hairy mole on a fair cheek is regarded often as an additional charm 123 124 . CONNECTIVE- f ISSUE TUMOUR^ rather than a disfigurement, if we can trust the taste of story-tellers, poets, and playwrights. Cervantes, describing the comic achievements of Don Quixote, makes the beautiful Dorothea describe her cham- pion as having on his right side, under the left shoulder, or somewhere thereabouts, a tawny mole overgrown with a tuft of hair not unlike that of a horse's mane. (Motteux's translation. Chap, xxx.) In the " Arabian Nights " the allusions are many ; thus the youth in the Eldest Lady's Tale saj'^s : " Persian poets have a thousand conceits in praise of the mole." Some of these allusions are certainly exquisite. Here is an example : " A nut-brown raole sits throned upon a cheek Of rosiest red beneath an eye of jet, English writers often refer to moles. Marlowe, in his powerful tragedy Dr. Faustus, when he causes Alexander and his paramour (Act iv., sc. 1) to appear before Charles, Emperor of Germany, makes the Emperor say : " I have heard it said That this fair lady, when she lived on earth, Had on her neck a little wart or mole.", There are numerous references to moles scattered in Shakespeare's plays. All who have read Cymbeline will remember the cunning use lachimo makes of the fact that Imogen had " On her left breast , A mole cinque-spotted, like the crimson drops I' the bottom of a cowslip." Cymbeline, when his lost sons, Guiderius and Arviragus, are presented to him in his tent, says : " Guiderius had Upon his neck a mole, a sanguine star ; It was a mark of wonder." To which Belarius replies : " This is he. Who hath upon him still that natural stamp It was wise Nature's end in the donation To be his evidence now." MOLES 125 In the Comedy of Errors, Dromio of Syracuse, in his comic account of the kitchen wench, tells his master that she knew what private marks he had about him, such as " the mole in my neck, the great wart on my left arm," etc. (Act iii., sc. 2). The occurrence of moles and other varieties of mother- rig. 67. — An extensive hairy mole. (From a jncture in the Museum of the iliddlesex Hospital.) marks has always been a subject of great speculation among matrons and the superstitious of all countries and all times. Peculiarly marked bull calves (apis bulls) were particularly venerated by the priests at Memphis, and when these bulls died they were accorded remarkable sepulchral rites. Moles are more particularl}'^ related to the " longings " of pregnant women, who believe that if these are not appeased the child will appear with the wished-for article, usually 126 CONNECTIVE-TISSUE TUMOURS flowers or fruit, marked on its skin. Though these matters receive no support from the scientific investigator, there is no belief more deeply rooted in the minds of matrons, 3"oung or old. The tradition comes to us from remote anti- quity, and the way in which Jacob turned it to advantage is well set forth in his crafty management of Laban's flock (Genesis xxx. 37). Fig. 68. — Extensive hairy mole upon the face of a boy a year old. The case of Esau who " came out red all over like an hairy garment" (Genesis xxv. 25), which curiously fascinates biblical commentators and matrons, had a parallel in a girl born at Pisa, hairy all over. In this instance, the mother attributed it to the fact that during her pregnancy she had gazed at a picture of John the Baptist. This is a good example of the circumstantial and plausible way women endeavour to account for these things. The belief even survives the ridicule of CONJUNCTIVAL MOLES 127 Charles Dickens, who represents Mrs. Gamp telling about a man six-foot-three " marked with a mad bull in Wellinofton boots upon the left arm," because his mother took refuge in a shoemaker's shop when frightened by a mad bull during her pregnancy (" Martin Chuzzlewit," Chap. 46). Hairy patches on the conjunctiva (conjunctival moles). — The mucous membrane (conjunctiva) on the ocular surface of the eyelids and adjacent portions of the eyeball occasionally presents a patch of skin which in appearance and structure resembles a hairy mole. Such a patch is called a dermoid pterygium. These dermoid patches occur most frequently at the margins of the cornea, and usually in the line of the palpe- bral fissure — that is, directly in the equator of the cornea ; but they are by no means confined to these situations. Usually they are limited to the conjunctiva covering the sclerotic, or trespass but little on the cornea. Sometimes, however, they involve a considerable extent of the corneal surface (Fig. 69). Wardrop described one in a man 50 years of age ; it was congenital. Twelve long hairs grew from its middle, passed between the eyelids, and hung over the cheek. These hairs did not appear until the sixteenth year, at which time the beard began to grow. Occasionally a mole will be found on each side of the cornea in the line of the palpebral fissure. A rare variety is limited to the caruncle : this is simply an excessive de- velopment of the delicate hairs that normally beset the caruncle (Fig. 70). These moles are occasionally associated with malforma- tions of the eyelids, especially the one known as coloboma of the upper eyelid. When this association occurs, the defect in the lid corresponds to the cutaneous patch on the conjunctiva. This combination is of some importance, as it is used as evidence in support of an explanation that has been put forward in regard to such hairy patches, based upon the development of the eyelids. In the embryo the tissue covering the outer surface of the eyeball, which ultimately becomes the conjunctiva, is directly continuous and in structure identical with the skin at the margin of the orbit. Very early, cutaneous 128 CONNECTIVE-TISSUE TUMOURS folds arise and gradually grow over the surface of the eyeball, and come into apposition at a spot corresponding to the future palpebral fissure. These folds ultimately become the eyelids. The surface of each fold, which is continuous with '^'^'MMim^ Fig. 6P. — Dermoid pterygium — common varietj'. the covering of the eyeball, becomes converted into mucous membrane, termed conjunctiva. In every normal eye the conjunctiva bears evidence of its transformation from skin, inasmuch as the caruncle at its inner angle is iur- nished with delicate hairs. It is reasonable to suppose that, as the occlusion of the proper covering of the eyeball by the eyelids is the cause of the conversion of the con- junctiva into mucous membrane, if from any cause a part, Fig. 70. — Excessive growth of hair on the caruncle, associated with an eccentric pupil. {After Semours.) or even the Avhole of it, were left uncovered, the exposed part would persist as skin. This is precisely what occurs. When the eyelid is in the condition of coloboma — a defect due, in all probability, to the imperfect union of the em- bryonic eyelid to the skin covering the fronto-nasal plate — DERMOID PTERYGIUM 129 a piece of conjunctiva persists as skin, and forms a mole occupying the gap in the Hd. Moles occur on the con- junctiva unassociated with coloboma, but in nearly every instance they are situated on the cornea in the line of the palpebral fissure. This circumstance would indicate that during development the conjunctiva was imperfectly covered by the developing lids. In a few very exceptional cases Fig. 71. — Dermoid pterygium in a sheep. the eyes have been found completely covered with skin without any traces of eyelids. Such a condition is known as cryptophthalmos, and the explanation offered concerning it is, that in these cases the eyelids have failed to appear, and in consequence the conjunctiva has jjersisted as skin. Conjunctival moles have been observed in horses, sheep, oxen, and dogs, and are furnished with hair or wool, according to the nature of the tegumentary covering of the mammal in which they occur. CHAPTER XII NEUROMAS AND ALLIED CONDITIONS OF THE NERVOUS SYSTEM Neuroma. — This may be defined as a tumour growing from, and in structure resemblino- the sheath of a nerve. The term neuromas is frequently used, especially in clinical work, as signifying tumours on nerves, but as such tumours are sometimes composed of fibrous, fatt}^ or even sarcomatous tissue, it would be better to speak of them as lipomas of nerves, sarcomas of nerves, and so on. The tumours which most strictly correspond to my definition are those known as neuro-fibromas, and it will be convenient to include the curious nodule known as the " painful subcutaneous tubercle." A neuro-fibroma is usually fusiform, and grows from the side of a nerve; when large, it may spread out the fasciculi of the nerve ; exceptionally the nerve-fibres will traverse the tumour. The long axis of the neuroma coincides with that of the nerve from which it grows. In size neuro-fibromas vary greatly : some are no larger than lentils, others may be as big as eggs ; larger specimens are very exceptional. They occur on the cranial as well as on the spinal nerves, and form on their roots trunks, branches, or the terminal twigs. Neuro-fibromas form smooth swellings, which are mobile, and when situated in the subcutaneous tissue glide easily under the skin ; they are encapsuled, may be easily enucleated, and are extremely liable to become myxomatous, and in large specimens this change leads to the formation of cavities in the tumours. These changes account for the various names applied to them, such as myxoma, myxo-fibroma, myxo-sarcoma, and the like. Painful subcutaneous tubercle. — This term was applied 130 NEUROMAS 131 by Wood in 1812 to a small discrete nodule which forms in the subcutaneous tissue. It is usually of the "size and form of a flattened garden pea," but it very rarely exceeds the size of a coffee-bean. When examined by the finger it feels like a small shotty body slipping about imme- diately beneath the skin. Structurally the " tubercle " con- sists of fibrous tissue very like that which constitutes the bulk of the nodules in molluscum fibrosum ; it is rare that a nerve-fibril can be traced to it. The interest of these bodies is due to the " very severe and excruciating pain" associated with them. The pain is paroxysmal, and usually increases in severity and in frequency according to the length of time the disease has existed. If the " tubercle " — for it is usually solitary — is struck, or even touched, acute pain is produced. They occur much more frequently in women than in men, and are commonly met with in early adult life; and though a " tubercle " may form on any part of the body, it shows marked preference for the lower limb. Excision of the little body at once, and permanently, arrests the pain. Ganglionic neuroma. — This is a tumour composed of nerve-cells, nerve-fibres, and neuroglia. They are extremely rare tumours. Klebs described a tumour of this kind which grew from the floor of the fourth ventricle near the calamus scriptorius. The tumour was nearly as large as a walnut. It has been thought that some tumours described as gliomas may have been ganglionic neuro- mas ; on the other hand, however carefully the histologic features of these tumours have been described, there has always been a doubt lest normal brain-tissue became included in the tumour. However, this cloud has been dispelled by the observation that tumours containing ganglionic tissues occur in connexion with the great cords of the sympathetic system as well as in the sub- cutaneous tisue. In one of the most remarkable cases, recorded by Knauss, a girl 8 years old had sixty-three tumours in the subcutaneous tissue of the trunk and thighs (Fig. 72); they varied in size from a pea to an orange, were firm 132 CONNECTIVE-TISSUE TUMOUSS and elastic, and not painful. Microscopically these tumours were found to be composed of ganglionic nerve-cells, Fig. 72. — Girl 8 years of age with sixty-three ganglionic neiiromas in the subcutaneous tissue of the trunk and thigh. {Knauss.) medullated and non-medullated nerve-fibres. Knauss believed that the tumours were derived from the minute ganglia QANGLIONIG NEUROMAS 133 on the finest terminal fibres of the sympathetic system which accompany the blood- and lymph-vessels. Knauss's description Fig. 73. — Multiple inoUuscum fibrosum. of the microscopic characters of the tumours, which clinically resembled lipomas, is accompanied by careful drawings. 13i G ONNEG TIVE- TISS UE TUMO UBS NEURO-FIBROMATOSIS Under this heading it is now necessary to describe several affections which were formerly regarded as being quite distinct. These are multiple neuromas, molluscum Fig. 74. — Native of Sierra Leone aged 50 years with molluscum fibrosum. The tumours, which were congenital, varied in size from a pepjjercorn to a billiard-ball. {Lamjrrey.) fibrosum, plexiform neuromas, sarcomas of nerves, and glioma. It will be useful to state a few facts concerning each of these conditions before describing their intimate relationship. It has long been known that neuromas sometimes NE UBO-FIBBOMATOSIS 13.: occur on nerves in extraordinary numbers. The remark- able case of Micliael Lawlor, described in Smith's classical monograph, was in all probability an example of this com- bination. It was estimated that this man had at least 2,000 tumours. There were 450 tumours counted on the nerves of the right lower limb, and 300 on the left. There were 200 tumours on the right and 100 on the left upper limb. The pneumogastric nerves and their branches Fig. 75. — Native of Bengal with molluscum fibrosum of the arm : there were also discrete nodules on other jparts of the body. The man belonged to the cow- keeper caste. {From a photofirajih sent hy Dr. Maddox, Bengal.) possessed 60 tumours, some of large size. The remainder were on the trunk. Several cases of this kind have been carefully described, but probably in no individual has a greater number of nodules been detected. In 1882 Professor von Recklinghausen published an im- portant monograph, in which he demonstrated not only that multiple neuromas were sometimes associated with molluscum fibrosum, but that the two conditions were closely related, and 136 CONNECTIVE -TISSUE TUMOURS he urged that the moUuscum bodies of the skin are formed on the cutaneous nerves, and are as truly neuromas as the tumours on the epineurium of the larger nerves. In typical cases of moUuscum fibrosum the skin of the trunk and limbs presents numerous small tumours, consisting Fig -Multiple moUuscum nodules on the scalp, nose, and fingers. The nodules on the fingers were ia the course of the digital nerves. mainly of fibrous tissue springing from the subcutaneous connective tissue. These tumours are of various sizes, some being no larger than a pin's head, whilst many are as big as a filbert, and a few even larger. The majority are about the size of a small pea. Many are sessile, and others are distinctly pedunculated, but all are covered with skin. These tumours MOLLTTSGUM FiBROSTJM 137 are mobile, soft to the touch, and of the consistence of firm fat. Sometimes the disease affects a broad area of skin on the head, trunk, or hmbs, causing it to hang in pendulous folds Fig. 77. — Molluscum fibrosum of the vulva in a negress. The tumour was removed : it weighed 75 lbs. The tumour had beau growing ten years : after its removal the patient conceived, and was delivered of twins in the bush. She died of puerperal fever. (Figs. 74, 75). Exceptionally the pendulous and nodular lesions occur in the same patient (Fig. 74). In its mildest form molluscum fibrosum appears as a single pedunculated tumour, a frequent situation being the labium majus (Fig. 77). 138 GONNEGTIVE-TISSVE TUMOURS The structure of these soHtary tumours is the same as the nodules in the muhiple forms and the pendulous skin-folds. An unusual situation is the mammary areola (Fig. 78) or the nipple. When these nodules grow from the nose they are apt to be confounded with the condition commonly but erroneously called "lipoma nasi." Concerning the cause of molluscum fibrosum, nothing is known. The disease is not confined to any climate or race, /, V /W^.Jc Fig. 78. — Pedunculated molluscum fibrosum from the nipple of a woman. [Museum, Middlesex Hospital.') for it has been observed in North America, the British Isles, India, Germany, and the West Coast of Africa. Under the term pachydermatocele, Mott (1854) described and figured several examples of the pendulous form of mol- luscum fibrosum which were successfully submitted to opera- tion, and the early volumes of the Transactions of the Pathological Society, London, contain descriptions and figures of this disease under a variety of names. The frontispiiece to Virchow's "Die Krankhaften Geschwtilste" is a representation of a woman with pendulous folds and a multitude of cutaneous nodules, under the title " fibrosum molluscum multiplex." The disease appears to be equally common in men and in women. SARCOMA OF NERVES 139 An important feature connected with the typical general- ized neuro-fibromatosis is the liability of the patients to sarcoma ; this may develop primarily, or arise as a malignant change in a molluscum nodide which has existed very many years. Sarcomas of this kind do not, as a rule, disseminate. In the generalized neuro-fibromatosis, death often results from gradual exhaustion, due to ulceration, septic changes, or sloughing of the pendulous portions of the skin. In many cases some intercurrent malady supervenes, such as pneu- monia ; in patients with multiple nodules on the roots of the spinal nerves, one of them may so enlarge as to press on the cord and produce fatal paraplegia. In regard to sarcoma supervening in the so-called mollus- cum nodules, it is necessary to remember that spindle-celled sarcomas arise primarily in nerve-trunks, especially in the great sciatic and its branches, quite apart from the existence of neuro-fibromatosis, localized or general. A sarcoma of a nerve recurs after removal or amputation of the limb, but dissemination is not frequent. Knauss, " Zur Kentniss der achten Neuroma." — Virchow's Areli., cliii. 29. Mott, Valentine, " Remarks on a peculiar form of Tumour of the Skin de- nominated 'Pachydermatocele'; illustrated by Cases." — Mecl.-CJdr. Trans., 1854, xxxvii. 155. Payne, J. F., "Multiple Neuro-Fibromata in connection with Molluscum Fibrosura." — Trans. Path. Soc, 1887, xxxviii. 69. von Recklinghausen, F., " Ueber die multiplen Fibroma der Haut, und ihre Beziehung zu den multiplen Neuromen." — Festschrift zu Rudolf Virchow dargehraclit, Berlin, 1882. Smith, R. W., " Treatise on the Pathology, Diagnosis, and Treatment of Neuroma," Dublin, 1849. CHAPTER XIII NEUROMAS AND ALLIED CONDITIONS OF THE NERVOUS SYSTEM (Continued) PLEXIFORM NEUROMA. The peculiar condition to which this term has been appHed is essentially a fibromatosis which is confined to a par- ticular nerve or plexus of nerves. Although it is a rare condition, a sufficient number of cases have been care- fully observed and recorded to enable a fairly complete account of the disease to be written. A plexiform neuroma, instead of forming a distinct tumour as in the case of the solitary neuro-fibroma, appears as if the branches of a nerve distributed to a particular area of the skin became enlarged and elongated. The overlying skin becomes stretched, thinned and raised over the thickened nerves, and is often pigmented, the usual colour being brown, like that characteristic of the hairy mole. Occasionally the skin is coarse and thick, as in the case of a moUuscum nodule. The tumour feels like a bag containing a number of tortuous, irreo-ular, vermiform bodies, soft to the touch and mobile. These bodies vary in thicloiess from a crow-quill to that of the thumb ; manipulation does not produce pain, though the lumps themselves are sensitive. When the skin covering the tumour is reflected these elongated bodies will be found to lie in the direction of the nerve distributed to the part. Thus, on the back they will run in a transverse direction (Fig. 79), whereas on the scalp they will trend to the vertex, and so on. When these thickened nerves are divided the enlarge- ment will be seen to be due to the presence of a gelatinous tissue, and the appearance of the cut surface reminds one of the umbilical cord. Microscopic examination shows that 140 PLEXIFOBM NEUROMA 141 tliis thickening is due to overgrowth of the connective tissue of the nerve-sheath, and especially that part of it known as the endoneurium — that is, the delicate connective tissue between the individual fibres of a nerve-bundle. The Fig. 79. — Plexif orm neuromafrom the back of a youth aged 19 years. The skiu was the seat of a brown haiiy mole. (Bmns.) enlargement is by no means uniform, so that the so-called multiple neuromas are really due to local irregularities in a diffuse overgrowth of the connective tissue of the nerve- sheath. Widely different opinions are held by equally competent observers in regard to the effects of these changes in the 142 GONNEGTIVE-TISSUE TUMOURS k 'M my Fig. 80. — Plexiform neuromas li^ affecting the roots of the chorda equina and anterior crui'al nerve. sheath upon the axis-c^dinders of the nerves. Some maintain that de- generation occurs, and others that they are not affected. This ques- tion requires careful investigation. The diffuse character of the en- largement in plexiform neuromas is well shown in a remarkable specimen preserved in the Middle- sex Hospital Museum (Fig. 80). A man 45 years of age was admitted into the hospital with well-marked paraplegia. At the post-mortem examination a large number of small nodules was found on the roots of the nerves. Many of the roots were so beset with them as to resemble strings of beads. In the cervical region a tumour as large as a nut had compressed the cord and produced paraplegia (Fig. 81). There was a neuroma as big as an orange on the anterior crural nerve ; there were smaller ex- amples on the branches of the lumbar plexus. When these nerve-roots are carefully ex- amined they present the an- nulated ap- pearance so characteristic of the root of the ipecacuanha plant, and it is clearly seen that Fig. 81.— The cervical segment of the cord represented in the preceding figure. A nodule on one of the cervical roots com- pressed the cord and led to fatal para- plegia. FLEXIFOBM NEUROMA 143 the nerve-roots are thickened throughout, and that the nodosities are local exaggerations. The details of this case were recorded by Sibley in 1866. Any nerve, cranial or spinal, is liable to this disease, but among the cranial set it shows marked preference for the vagus and the trigeminus. It may affect parts of several nerves, or be limited to certain branches of a single nerve. The roots of nerves and terminal twigs may be attacked as well as their trunks ; and the branches of nerves within the muscles may display nodosities. The sympathetic nerves do not escape, for the great lateral cords as well as the visceral branches may be nodular with this disease, (Alexis Thomson.) Fig. 82. — Arm in which the musculo -spiral nerve and its branches were transformed into a plexiform neui'oraa. (After Camphell cle More/an.) In one instance the nerves involved included the facial hypoglossal, motor portion of the fifth and its lingual branch. The enlargement of the lingual and hypoglossal nerves produced macroglossia in a child aged 4 years, for which Abbott excised the protruding part of the tongue. Shattock investigated the diseased organ, and the outcome was an admirable paper of great value and interest. As examples of the disease limited to part of a nerve, reference may be made to some cases in which the ophthal- mic division of the trigeminus has been affected, leadino- to enlargement of the upper eyelid and proptosis, which neces- sitated excision of the eyeball, in one patient with fatal consequences (Friedenwald, Rockliffe and Parsons, Treacher Collins and Batten). 144 CONNECTIVE-TISSUE TUMOURS I have seen a plexiform neuroma strictly limited to the great occipital nerve. The scalp covering the affected nerve was transformed into a brown mole. Humerus. Musculo-spiral ntrve. Brachio-radialis muscle (supinator longus). Neuroma. Neuroma on the cuta- neous branches of the musculo -spiral nerve. Fig. 83. — The arm represented in the preceding figure dissected : the musculo-spiral nerve and its branches are transformed into a plexiform neuroma. In the limbs any nerve may be attacked, and the disease is usually limited to one nerve, and follows it out to its final ramification. One of the most remarkable specimens known is pre- PLEXIFOBM NEUROMA 145 served in the museum of the Middlesex Hospital; in this the musculo-spiral nerve is affected. The patient, a girl of 15 years, suffered amputation of the arm by Campbell de Morgan. (Figs. 82 and 83.) The musculo-spiral nerve is as thick as the thumb ; it looked gelatinous, like an umbilical cord. The cutaneous branches of the nerve are very thick and irregularly nodulated. The microscopic changes in the musculo-spiral nerve are identical with those found in the thickened nerves of a plexiform neuroma underlying the pigmented mother- marks. An interesting feature of this specimen is the large, smooth, ovoid tumour which occupies the bend of the arm, and is attached to one of the branches of the musculo- spiral nerve. Clinical features. — Neuromas are in the majority of cases innocent tumours ; they very rarely recur after com- plete removal {see Sarcoma of Nerves, p. 70). In exceptional environment a neuroma will cause death, and many examples have been observed in which even small neuromas on the roots of spinal nerves have produced paraplegia with a fatal ending (Fig. 81). Smith refers to a woman who complained of severe pain in the course of the right trigeminal nerve ; this pain was so much increased by mastication that she ate but little, and speaking aggravated it to such a degree that she remained silent unless interrogated, and even on these occasions she often preferred to reply by signs. She died after enduring severe and uninterrupted pain during four and a half months. At the autopsy a neuroma as large as a walnut occupied the situation of the right Gasserian ganglion. With modern methods of surgery no person would be allowed to suffer in this awful manner. The pain produced by the painful subcutaneous tubercle has already been mentioned. When a neuroma involves the roots of a spinal nerve, pain is a prominent symptom until the tumour is big enough to compress the cord and pro- duce paraplegia : these signs are not peculiar to neurojiias of the roots of the spinal nerves. Neuromas on the nerves of the limbs are usually solitar}^, ovoid ; the long axis of the tumours coincides with that of the limb and produces pain ; when pressed the painful sensations radiate throughout the K UQ GONNEGTIVE-TISSUE TUMOURS distribution of the nerve below the point of attachment of the neuroma. In a remarkable case recorded b}^ Semon, an ovoid tumour, in all probability a neuroma of the internal branch of the superior laryngeal nerve, projected into the ventricle of the larynx of a woman 40 years of age. The tumour was noticed in 1888, and it caused very little trouble except when pressed or handled (then coughing and retching occurred immediately) until 1891, when it was necessary to perform tracheotomy. In 1904 Semon removed the tumour through an incision in the neck, with success, following the plan adopted by Paul von Bruns in a similar case, which occurred in a boy of 13 years. Treatment, — A solitary neuroma in an accessible position is easily removed, care being taken during the enucleation not to damage the fibres of the nerve. It sometimes happens that the neuromatous nature of a tumour is not recognized until after its removal with a segment of the nerve. In the limbs, such breaches in the continuity of a nerve-trunk have been repaired by grafting fragments of nerves from ampu- tated limbs, or from dogs and rabbits ; it is, however, always better to avoid this accident by careful surgery than to remedy it by secondary measures, however brilliant. Per- sistent facial palsy has followed the removal of a neuroma lodged in the parotid gland. A neuroma Avithin the spinal canal has been successfully excised. Multiple neuromas, especially when associated with mol- luscum fibrosum, are beyond the art of surgery. Plexiform neuromas have been several times successfully excised : exceptionally, when affecting a limb, amputation has been found necessary. This form of neuroma in the nerves of the tongue has produced enlargement of the tongue re- sembling macroglossia : the condition was remedied by excision of a portion of the tongue. Friedenwald, Harry, " A Case of Plexiform Neuroma of the Eyelid (Ranken- iieurom)." — Jolins Hopkins Hosp. RejHs., 19C0, ix, 355. de Morgan, Campbell, " Case of Multiple Neuroma of the Forearm." — Trans. Path. Soc, 1S75, xxvi. 2. Semon, Sir Felix, " Soft Fibroma of the 'L^vjms.."—Brit. Med. Jonrn., 1905, i, tt. BEFEBUNGES 147 Shattock, S. G., and Abbott, F. C, " Macrogiossia Neuro-Fibromatosa." — Trans. Path. Soc, liv. 231. Sibley, Septimus W., " A Case of Multiple Neuromata affecting the Nerves both within and external to the Spinal Canal." — 'Med.-Chir. Trans., 1866, xlix. 39. Thomson, H. Alexis, " Neuroma and Neuro-Fibromatosis." — Edinburgh, 1900. Woo6i.~Min. Med.and Snrg. Journ., 1812, p. 283. CPIAPTER XIV NEUROMAS AND ALLIED CONDITIONS OF THE NERVOUS SYSTEM (Conclwded) GLIOMA OF THE BRAIN; OF THE RETINA AND OPTIC NERYE ; AND OF THE SPINAL CORD Glioma of the brain. — Ever since I became practically acquainted with the changes in the nerves constituting a plexiform neuroma, it seemed to me that they were akin to the localized neuroglia overgrowth in the brain known as glioma, and I was sufficiently convinced of this to draw attention to the likeness in the first edition of this mono- graph (1894). A glioma of the brain occurs as a translucent swelling imperfectly demarcated from the surrounding parts ; the gliomatous tissue may have the consistence of the vitreous, or be as firm as the tissue of the pons. Microscopically it has the characters of an overgrowth of neuroglia. Yirchow pointed out that when a glioma is situated near the surface of the cerebral cortex it appears like a colossal convolution. Should it grow in the tissue of an optic thalamus, this structure will bulge into the third ventricle as though overgrown, and a glioma of the occi- pital lobe will project into the descending cornu like an additional thalamus. The best illustrations of this in- definiteness, so characteristic of gliomas, come out very strikingl}^ when the pons and the cerebral crura are occu- pied by this form of tumour. A glioma occasionally occurs in the pons, and forms a tumour of considerable size (Schorstein). It may be confined to one side, and extend into the adjacent cerebellar crura. In a case described by Cayley, which occurred in a child 2 years of age, a glioma as large as a walnut occupied the right half of the pons and extended along the superior 148 GLIOMAS 149 cerebellar peduncle of that side, reaching as far forwards as the corpora quadrigemina. The gliomatoiis mass formed a prominence on the corresponding half of the floor of the fourth ventricle, and obstructed the Sylvian aqueduct. In some cases both sides of the pons are involved, and the overgrowth of neuroglia extends forwards into the cere- bral crura and the cerebellar peduncles, and involves the Fig. 84. — Bilateral gliomatous enlargement of the pons and crura cerebri. {Angel Mo)ieij.) corpora quadrigemina. In a few it extends downwards into the medulla, and may even involve the cervical portion of the cord (Whipham). Sometimes the gliomatous tissue is so abundant as to produce an enlargement of the pons and cerebral peduncles. (Fig. 84 ) The appearance of such brains is very peculiar : the basilar artery and its branches appear as though sunk in deep furrows, Avhich cause the parts to resemble " a soft 150 GONNECTIVE-TISSVE TUMOURS package tightly corded " (Dickinson). Such cases are rare, and in nearly all instances the patients have been under 12 years of age (Percy Kidd, Gee, Angel Money, and Goodhart). A case has been observed in a man of 32 years (Schulz). The relations of a glioma to surrounding tissues are best seen in recent specimens. On examination soon after death the diseased parts are found abnormally large, and on section exhibit a characteristic pale-blue colour ; in thin sections the tissue has a delicate trans- lucent appearance. The tumour itself is very soft, and imparts to the fingers a sensation like fluctuation. When the parts are immersed in alcohol the tissue becomes firm, opaque, and white; under these conditions it is particu- larly difficult to determine the limits of the tumour. Gliomatous tumours of the brain are purely local ; their growth appears to be limited by the cerebral membranes, and they do not disseminate. They vary greatly in structure, for some consist mainly of round cells and others are com- posed of spider cells ; in some the cells are spindle-shaped. They are in no way related to sarcomas. Sarcoma of the optic nerve. — Tumours of the optic nerve are very rare. A careful analysis of recorded cases does not afford much clear information on the pathologic aspect of these tumours, and they are described under titles such as glioma, myxoma, myxo-sarcoma, fibroma, and sarcoma. The recorded clinical facts are sufficient to prove that tumours of connective tissue with malignant characters do arise from and in the optic nerve. They are unilateral, and more frequent in the young than in adults. The greater proportion are met with before the age of 20, and of these by far the larger proportion occur before the tenth year of life. The optic nerve is a complex structure, and in the embryo it is preceded by an outgrowth from the brain known as the optic stalk ; this is hollow, and consists of epithelial cells. This stalk is ultimately replaced by a fibrous nerve, the nerve elements of which are in part derived from the retina and in part, perhaps, from the brain (Robinson). Thus the early tissue of the optic stalk GLIOMAS 151 is identical in structure and continuous with the susten- tacular tissue of the embryonic retina. These facts are of importance because in some cases, especially in adults, sarcomas arise from the sheath of the nerve, and do not primarily involve the nerve-fibres. Pockley has excised a tumour from the optic nerve, and saved the nerve and the globe. The patient was a boy ot 19 years. The tumour is described as an encapsuled round- celled sarcoma. Some recently recorded cases which have been very carefully investigated point to the conclusion that many of the tumours arising within the sheath of the nerve, especially in children, are particularly connected with the pial sheath, and in construction are closely allied, if not identical, with the retinal sarcoma (glioma) of infancy. The malignancy of optic-nerve sarcomas, though pronounced, is not excessive. Tumours of the optic nerve are usually ovoid in shape, with the long axis coincident with that of the nerve. Their surfaces are usually smooth, and in size they vary greatly, but rarely exceed a pigeon's egg. They do not tend to invade the globe, but are apt to creep through the optic foramen and involve the intracranial portion of the nerve. As the fibres of the nerve are early implicated, vision is soon interfered with ; there is proptosis, but the movements of the eye are free, and there is no pain, even on manipulation. Much of the confusion relating to the nomenclature and structure of tumours of the optic nerve is due to their rarity, and those interested in this question will do well to study the careful work of Treachei- Collins and Devereux Marshall. Glioma of the retina. — In structure this tumour mimics the granular layer of the retina, and Treacher Collins has drawn attention to the great similarity which exists between the cells composing the retina of the foetus at the third month, when its layers are undifferentiated, and the tissue of a retinal glioma. This tumour occurs exclusively in children ; exception- ally it has been detected at birth, more commonly it makes its appearance during the first four years of life ; 152 CONNECTIVE-TISSUE TUMOURS it is very rare after the seventh year, and is almost un- known after the age of 12. In a certain proportion of cases (20 per cent.) both retinae are affected, either simul- taneously or after a brief interval. This is always an indication that the tumour is highly malignant. In the early stages there is usually no pain or symptom denoting the presence of a tumour ; gradually the pupil dilates, and a peculiar reflex is noted at the fundus (this is often termed cat's-eye), and, under test, the eye will be found quite blind. As soon as the existence of a glioma is discovered by the surgeon, the eye is, as a rule, promptly excised. In cases where treatment of this kind is refused or deferred, the following changes occur. The tumour^ continuing to increase, pushes forward the intra-ocular structures and causes great pain as the " result of the in- creased intra-ocular pressure it produces, until the cornea fields and the tumour bursts forth, and, growing very rapidly, soon makes its way between the eyelids, which become swollen and everted; and then, in consequence of exposure, it assumes a dusky red fleshy appear- ance, whilst from its surface a sanious fluid exudes which may form crusts on the surface of the tumour. Should the parts become excoriated or handled, they bleed freely. A fungating tumour of this kind will sometimes attain a very large size before it destroys the child's life. After excision of an eye for retinal sarcoma the dis- ease is very prone to recur, and the recurrent tumour may attain very large proportions before it destroys life. When the operation has been long delayed the growth may have burst through the sclerotic and invaded the orbital tissues ; in a larger proportion of cases it has infil- trated the optic nerve, and it is in this structure that the disease reappears. The frequency with which sarcoma returns in the stumjD of the optic nerve is, in all prob- ability, due to the intimate lymphatic relations of this nerve with the intra-ocular lymph-spaces. In regard to the question whether "glioma" may ''run" in a family, there is little evidence to guide us. Fuchs has recorded a case in which two children were affected in one family, and two very extraordinary reports have recently GLIOMAS 153 come from Australia. Earle Newton states that in a family of sixteen children ten died from retinal glioma ; three of the cases were unilateral and seven bilateral. All the affected children, with one exception, died about the third year. Maher tells of a family of four children, of whom three died of glioma, and in two it was bilateral. Dissemination of retinal sarcoma is exceptional. The common situations for secondary deposits are the brain, the lymph-glands about the jaws, and the periosteum of the skull-bones. The treatment for retinal sarcomas is removal of the eye, and the importance of promptness in this matter is indicated in the careful inquiry conducted by Lawford and Collins. They prove very clearly the following points : — The quicker an eye is removed after the discovery of the disease, the better the prospect of cure. In the majority of cases the disease returns in the orbit, and in a very small proportion of cases secondary deposits occur in other parts. When recurrence takes place it is rarely delayed beyond nine months ; but one undoubted case has been reported in which the disease returned three years after the primary operation. If three years elapse and there is no recurrence, the recovery may be regarded as permanent. Out of fifty-four cases in Lawford and Collins's list, eight patients were alive and free from recurrence three years after the removal of the eye for retinal glioma. It is significant to note that in seven of these cases the disease affected one eye only. This shows the almost hopeless condition of the patient when both eyes are affected. Other statistical inquiries have been conducted with the view of obtaining the percentage of cures in this disease, and they work out at about the same proportion as in the paper mentioned above. Glioma of the spinal cord. — A glioma of the spinal cord is a very rare tumour, and, judging from the scanty records, it would appear that a glioma in the brain is twenty times more frequent than in the cord. The tumour is im- perfectly demarcated from the nervous tissue, and often causes a general enlargement of the cord, producing upon it an effect like gliomatous disease of the pons, crura, and 154 G0NNEGTIVE-TI8SUE TUMOURS medulla. It was pointed out in connexion with this disease of the medulla that it sometimes involves the adjacent segment of the spinal cord. Resinger collected and epitomized the records of nineteen cases of glioma of the spinal cord, and added a full descrip- tion of a case which he observed ; the report is accompanied by an account of the morbid anatomy of the parts by Prof Marchand. The disease may attack any part of the cord, but is most frequent in the cervical enlargement. In a few instances the tumour was seated in the lumbar region. It appears most frequently between the seventeenth and thirtieth years, but it has been observed as late as 50. Sharkey has published an interesting account of a spinal glioma which occurred in a man 50 years old, and he uses it to demonstrate the clinical fact that when a tumour arises within the cord, as, gliomas always do, it disturbs its functions from the commencement ; but, as the nerve substance appears to be elastic, and to allow a good deal of gradual stretching without serious interference with its functions, a tumour may continue to grow for a long time before it produces striking pathologic phenomena. When a tumour grows in the spinal canal outside the cord it may produce but few symptoms until it presses the cord against the resisting Avails of the canal ; after this has taken place the course of the disease is naturally very rapid, as the cord is quickly flattened by the constantly increasing demands for growing-space which are made by the tumour. The peculiar relation of the gliomatous tissue to the nerve- tissue of the cord precludes any surgical interference. Cayley, W., " Gliomatous Tumours of the Brain." — Trans. Path, Soc, 1865, xvi. 23. Collins, Treacher, " Anatomy and Physiology of the Eye," 1896, p. 84. Gee, S., " A Second Case of Gelatiniform Enlargement of the Pons Varolii."— St. Bart.'s Hosp. Mepts., 1881, xvii. 287. Groodhart, James F., " Cases of Cerebral Tumour." — Trans. Path. Soc, 188G, xxxvii. 14. Kidd, Percy, "A Case of Great Enlargement of the Pons, Crura Cerebri, and Medulla."— ,%. Bart.'s Hasp. Mepts., 1877, xiii. 271. Lxwford, J. B., and Collins, E. Treacher, " Notes on Glioma Retinse, with a report of GO Cases."— i^^'y. Land. Ophthal. Eosp. Rejrts., 1893, xiii. 12. BEFEBENGES 155 Maher, Anstralaslan Med. Gac, 1902. Marshall, Devereux, " Further Note on a Case of Optic Nerve Tumour pre- viously reported to the Society." — Trans. OphtTial. Soc, 1900, xx. 164. Marshall, Devereux, " Implantation Cysts of the Iris." — Trans. OjiJdhal. Soc, 1899, xix. 54. Money, Angel, " Gliomatous Enlargement of the Pons Varolii in Children." — MeoL.-Chir. Trans., 1883, Ixvi. 283. Newton, Earle, Australasian Med. Gaz., 1902. Pockley, Australasian Med: Gaz., Oct. 1901. Resinger, " Ueber das Gliom des Riickenmarkes, Beschreibung eines Hirner- gehorigen Falles, mit anatomischer Untersuchung von Prof. Marchand." — Virchow's AreJi. f.path. Anat., 1884, xcviii. 3G9. Schorstein, G., and Watson, A. J., "A Case of Glioma of the Pons." — Lancet, 190G, i. 1035. Schulz, Richard, " Gliomatose Hypertrophie des Pons und der Medulla Oblongata." — ■Neuroloijisches Centralbl., 1883, ii. 5. (This paper con- tains several references.) Sharkey, S. J., " Spasm in Chronic Nerve Disease." — Guhtonian Lectures, 188G, pp. 53-58. Whipham, T., " Tumour (Glioma) of the Spinal Cord and Medulla Oblongata : Dilatation of the Lymphatics ; Large Cavity occupying the position of the Central Canal (Syringomyelus)." — Trans. Path. Soc, 1881, xxxii. 8. CHAPTER XV ANGEIOMA AND LYMPHANGEIOMA ANGEIOMAS An" angeioma is a t-umour comjiosed of an abnormal forma- tion of blood-vessels. This genus contains three species : 1 . Simple ncevus. 2. Cavernous noevus. 3. Plexiform angeioma. 1. Simple naevus. — This is the most common species of nsevus, and in its typical form affects the skin and sub- cutaneous tissue. A ngevus may aj)pear as a superficial discoloration of the skin, and is either a lively pink or a deep blue : these are known as " port-wine stains." Such nsevi may involve an area of skin 2 cm. scjuare, or extend over a large portion of the face, or half the trunk, or be restricted to a limb. James II. of Scotland had a stain on one cheek and was called "Fiery-Face." A very common variety of nsevus is that often referred to as telang-eiectasis; it consists of an abnormal collection of arterioles situated in the skin and subcutaneous tissue; it may be present at birth, but much more frequently appears in the course of the first few weeks of life. Sometimes a nsevus appears as a red spot no larger than a split pea; then suddenly it grows actively, and 'in two or three months will involve an area of skin 4 cm. square. When the nsevus consists mainly of arterioles it will be bright pink; when composed mainly of venules it will be of a bluish tint. Lymphatics are often present. Structurally, nsevi are com- posed of minute blood-vessels embedded in fat; usually two or more large vessels establish a communication between the nsevus and an adjacent artery or vein. The vessels 156 ANGEI0MA8 157 of the nfevus are often sacculated. When gently compressed, the blood is driven from the nsevus, which at once loses its colour ; but the colour returns as soon as the pressure is relieved. Simple nffivi are common enough in the skin of the face, scalp, neck, and back. They are less frequent on the limbs. They also occur on the labia, the lips, tongue, and conjunctivae. NtBvi of small size frequently disappear spontaneously ; more often they gradually increase in size, and may become converted into cavernous nyevi, or endotheliomas (Chap. xli.). 2. Cavernous nsevus. — This is the species to which the term erectile tumour is most applicable. In structure it is comparable to the spongy tissue characteristic of the cavernous tissue of the penis. Cavernous, like simple na^vi are most frequently seen in connexion with the skin, where they form distinct tumours of a red or blue colour, rising- above the general surface ; sometimes they display the peculiar tint so characteristic of fluid contained in thin- walled cysts, for which a cavernous nsevus is often mistaken, especi- ally when situated near the outer angle of the orbit. In most cases the blood can, by firm and steady pressure, be squeezed out of a nsevus, but the swelling quickly reappears after the compression is removed. The surface of a nsevas may feel warmer than the surroundinof skin, and sometimes the tumour pulsates, the movement being appreciable to the finger, and occasionally perceptible to the eye. Structurally, cavernous na^vi are made up of variously shaped spaces and sinuses, the walls of which are merely fibrous septa, lined with endothelium. Some of these nsevi consist in part of vessels and in part of cavernous spaces. When an angeioma consists entirely of irregular blood-con- taining spaces, a dissection round its periphery will reveal the existence of vessels, sometimes of considerable size, conveying blood to it from adjacent arteries. Cavernous, like simple ntevi are, as a rule, congenital, but a nasvus which during infancy is small and inconspicuous may later in life become converted into a cavernous nsevus of lars^e size, and one that will, under certain conditions, jeopardize life. Verj^ large cavernous mevi have been observed in the breast, in the male as well as in the female. 158 CONNECTIVE-TISSUE TUMOURS Cavernous nsevi occasionally occur in tlie tongue ; as a rule, they are situated near the surface, and form slightly elevated patches of a deep-blue or livid colour. Such nsevi rarely give rise to any difficulty in diagnosis : their colour, general apj^earance, and the fact that firm pressure suffices to drive the blood out of the tumour are sufficient to indicate their ntevous character. Many lingual nrevi are congenital, but a fair proportion originate late in life. It must also be borne in mind that a small and inconspicuous naevus may, as years run on, develop almost silently into a dangerous erectile tumour. In some instances lingual nsevi cause very little incon- venience unless they bleed, but this accident may arise at any time, either by abrasion from hard food or from accidental bites, or in consequence of rubbing against jagged teeth. Under such conditions the ha3morrhage is sometimes very alarming, and so oft-repeated that it is in some instances imperative to excise the implicated half of the tongue. Except in the tongue and rectum, cavernous nsevi are very rare in mucous membranes. Cavernous angeiomas are sometimes found in voluntary muscles. Several interesting examples were collected and described by Campbell de Morgan in 1864 Examples have been observed and carefully recorded in the following muscles : the semimembranosus, semitendi- nosus, and deltoid ; and Eve has removed one involving the triceps and anconeus. The museum of the Royal College of Surgeons contains an example removed by Stonham from the gracilis. Rau has described a cavernous angeioma which occupied the wall of the right auricle ; the patient was 56 years of age, and the tumour equalled in size a small cherry, and occupied the deep layers of the endocardium. Cavernous angeiomas are of very rare occurrence in the larynx; nevertheless they have been observed in this situation, and the careful descriptions of some of the cases place the nature of the tumour beyond doubt. They have been observed springing from the vocal cords (Percy Kidd), the ventricular bands, and from the ventricle. The most striking examples arise in the sinus pyriformis. Usually such ANGE10MA8 159 tumours are sessile, but they are occasionally pedunculated ; they may be bright-red or purple. Laryngeal angeiomas may be smooth or nodulated like a mulberry ; they are rarely larger than a haricot bean. The colour of these tumours is the most striking clinical feature. An extremely rare situation for a cavernous navus is the subperitoneal tissue (Lane) ; another is the synovial membrane of the knee-joint, simulating tuberculous disease of that joint (Eve). The liver is not an unusual situation for cavernous npevi of small size. Nsevi are not uncommon in the livers of cats and feline mammals in general, but they appear to be harmless tumours. 3. Plexiform angeioma. — The angeiomas which will be included under this denomination are those usually designated as " aneurysms by anastomosis," or " cirsoid aneurysms." The former term appears to have been intro- duced by John Bell, but the expression "aneurysm by anastomosis " has come to be used so vaguely that its suppression is a matter of necessity. A plexiform angeioma consists of a number of abnormal blood-vessels of moderate size arranged parallel to each other, as in the rete mirabile of the fore limb of the sloth or the tail of a spider monkey. Such angeiomas may consist of arteries only' (arterial retia), or of veins (venous retia), or of arteries and veins in equal proportions (duplex retia). In some the vessels are very tortuous, a disposition more common with arteries than veins. Tortuous vessels are not infrequent in retia — for example, the arterial retia in the intercostal spaces beneath the pleura of cetaceans, and the rete in the pituitary fossa of oxen and sheep ; and the renal glomerulus. Plexiform angeiomas are very rare ; the largest that has come under my notice occurred in the perineum of a lad 19 3'ears of age : the corpus spongiosum was sur- rounded by a number of arteries as large as the coronary branches of the facial, and veins as big as the cephalic. The arrangement resembled that of a duplex rete. Miiller has recorded very carefully the clinical history and an account of the subsequent dissection of a very 160 GONNEGTIVE-TISSUE TUMOURS unusual example of plexiform angeioma. The patient, a man of 20 years, stated that his parents noticed a red spot on the left half of the forehead when he was a year old ; this gradually increased in size, and at the age of 12 it had become an obvious tumour. When the patient was 16 it not only grew rapidly, but began to " buzz." At the age of 20 the tumour exhibited all the characters Fig. 85. — Dissection of a plexiform angeioma of the forehead. (j4fter H. Mullo:) of a plexiform angeioma, the pulsation being attended by a whirring sound. P. Bruns ligatured the right external carotid and the left common and external carotid. The patient became hemiplegic on the second, and died on the third day after the operation. Death was due to embolism and thrombosis of the left middle cerebral artery. The parts were injected and dissected (Fig. 85) ; the angular arteries were large and very tortuous. Plexiform angeiomas occur in connexion with the ANGEIOMAS 161 cerebral arteries. They have been observed on the surface of the right anterior lobe of the cerebrum, fed mainly by the anterior and middle cerebral arteries. In two cases reported by Drysdale, one patient was a lad 17 years of age, and the other a woman aged 26 years. The woman was an epileptic. In another patient, a man aged 20, the angeioma was situated over the angular gyrus: the patient died from hoemorrhage from the tumour, which produced the typical signs of pressure on the motor region of the cortex (D'Arcy Power). A cavernous angeioma has been observed in the temporo-sphenoidal lobe of the brain of a male epileptic idiot aged 8 (Dobson). Treatment. — Nsevi come under observation almost daily ; in such cases it is usual to watch the child in order to ascertain whether the nasvus is growing or not: manynsevi disappear ; but when they become active and grow, they need prompt treatment. No method is so safe and effectual as excision, whenever it can be carried out, remembering always to cut the ncevus out, not cut into it. I have excised nsevi, simple and cavernous, from the skin over an unclosed fontanelle, the eyelids, the tongue, labium, and other parts of the body in more than one hundred chil- dren, and never had the least untoward symptom. It is infinitely preferable to treatment by electrolysis, nitric acid, ethylate of sodium, or the ligature. The chief reason for excising n^evi when they evince signs of activity is to prevent them from assuming such proportions as to pass beyond the limits of justifiable surgery. Many examples have been recorded in which a nsevous fleck in an infant has become a formidable tumour in the adult. The nsevi which are known as " stains " disappear under the influence of radium, It is impossible to advise in regard to the treatment of plexiform angeioma. Each case exhibits special features which will modify the operation, and the particular ]nethod employed will depend on the enterprise, experi- ence, and skill of the surgeon in charge of the case. Several cases of plexiform angeioma of the limbs have been recorded in which it has been necessary to resort to amputation. When the leg is involved this operation is L 162 CONNECTIVE-TISSUE TUMOURS attended with unusual risk of life. The operative difficulties and dangers in connexion with large plexiform angeiomas of the head and orbit are very great. LYMPHANGETOMAS A lymphangeioma has the sanae relation to lymphatics that an angeioma bears to hsemic capillaries. There are three species of lymphangeiomas : 1. Lymphatic nsevus. 2. Cavernous lymphangeioma. 3. Lymphatic cyst. 1. Lymphatic nsevus.— This species of lymphangeioma is, as a rule, colourless, but when it contains a fair number of h^emic capillaries, then the nsevus appears as a pale pink patch slightly raised above the level of the surrounding skin. When composed entirely of lymphatics it is yellowish-white ; when it is pricked, lymph (sometimes mixed with blood) issues from it. Occasionally several nsevi occur in the same individual ; they vary greatly in size — some are as small as shot, others may have a diameter of 2 cm. or more. In many instances they are noticed a few months after birth ; occasionally they seem to be acquired. This is probably explained on the ground that during infant life they are small, and their want of colour saves them from detection until their increase in size later in life makes them conspicuous. Lymphatic nsevi may occur in the skin on any part of the trunk or limbs, and have been especially studied in the mucous membrane of the tongue and lips. In connexion with the tongue the affections may be localized to a definite area and give rise to a lingual lymphangeioma ; this takes the form of a pale - pink papilla, or clusters of smooth papillae, projecting from the mucous membrane. Sometimes one half of the dorsum of the tongue will be beset with small rounded projections. These projections consist of clusters of dilated lymphatic vessels. There is a very rare disease of the tongue to which the name macroglossia is applied. Clinically the condition L YMPHANGEIOMAS 163 manifests itself" as a congenital enlargement of the tongue implicating mainly its anterior two-thirds. As the child grows the tongue increases so disproportionately that the mouth accommodates it with difficulty, and at last the tip of the organ protrudes from the mouth and, in severe examples, becomes so big as to extend far beyond the margins of the lips (Fig. 86). Fig. 86. — Macroglossia in a girl aged 11. {After Humphrij.) The increase in the size of the tongue is not due to an overgrowth of its muscular substance, but is caused, as Virchow pointed out, by the formation of a lymphangeioma in connexion with the lingual mucous membrane. Recent observations have shown that there is another cause of macroglossia, namely, plexiform neuroma affecting the lingual and hypoglossal nerves (p. 143). 2. Cavernous lymphangeioma. — This species in its 164 GONNEGTIVE-TISSVE TUMOURS naked-eye cliaracters resembles a lympliatic nseviis, but on microscopical examination it will be found to be identical in structure with the cavernous nsevus, with the difference that its cavities are filled with lymph instead of blood. Treatment. — -This is conducted on the same lines as for angeiomas. In the case of macroglossia, excision of the enlarged and protruding parts of the organ has been followed by permanent good consequences. Fig. 87.— Lymphatic cyst of the neck iu a child 2 years of age. 3. Lymphatic cyst. — This appears as a congenital swelling in the neck, axilla, and adjacent parts of the thoracic wall ; it was formerly classed under the title "hydrocele of the neck." Lymphatic cysts are easily recognized. They are always congenital ; even at birth they are sometimes of very large size, and exhibit a- preference for the anterior triangle. In some instances they extend into the axilla and superior medi- astinum, or project into the posterior triangle (Fig. 87). Their upward limit is, as a rule, indicated by the hyoid boue, but they have been known to reach as high as the parotid LYMPHATIC CYSTS 165 gland. The cyst may be unilateral or bilateral ; it may consist of a single cavity, or be multilocular and the various chambers may intercommunicate. In size they vary greatly : J3utte|=vA Fig. 88. —Child with a lymphatic cyst on the side of the thorax which probably arose m an angeioma. 166 CONNECTIVE-TISSUE TUMOURS some equal a fist, others are bigger than the head of the patient. When the walls of the cyst are thin and the over- lying skin is stretched, the tumour is as translucent as a thin-walled hydrocele of the tunica vaginalis testis. These cysts originate below the deep cervical fascia, but a portion may make its way through this membrane and become subcutaneous. Perhaps the most remarkable fact in connexion with them is the tendency they exhibit to shrivel and dis- appear ; they are exceptionally liable to inflame, and several cases have been recorded in which the cyst has been ruptured by the child falling upon it. Their proneness to spontaneous cure explains the rarity of such cysts after puberty. It has been many times observed that the spontaneous effacement of these cysts is preceded by a sudden increase in their size ; they become hot, tender, and pass into a state of inflammation, and as this subsides the cysts slowly disappear. The walls of lymphatic cysts are often composed of tissue so vascular as to merit the term nsevous ; it is probable that some of them have arisen in large cavernous nsevi which have been converted into cysts (Fig. 88). It is important to remember that lymphatics are often very abundant in the ordinary forms of cavernous nsevi. It is also a fact of some interest that a lymphatic cyst in the neck and well-marked macroglossia have been observed in the same individual. Barker, A. E., "Cavernous Nsevus of the Eectum proving Fatal, in an Adult, from Hsemorrhage." — Med.-Chir. Trans., 1883, Ixvi. 229. Barker, A. E., "A Case of Macroglossia, so-called Lymphangioma Caver- nosum." — Trans. Path. Soe., 1890, xli. 77. Bland-Sutton, J., " A Case of Erectile Tumour of the Male Breast." — Trans. Clm.Soc, 1889, xxii. 187. Dobson, Margaret B., "A Cavernous Angioma of the Temporo-Sphenoidal Lobe of the Brain."— ^ri^. Med. Journ., 1907, ii. 144. Drysdale, J. H., " Angioma Arteriale Racemosum : Two Cases." — Trans. Path. Soc, 1904. Iv. 66. Griffiths, J., " Case of Villous Sarcoma of the Neck and Heart." — Trans. Path. Soc, 1888, xxxix. 311. Humphry, G. M., "Hypertrophy and Prolapse of the Tongue." — Med.-Chir. Trans., 1853, xxxvi. 113. BEFEBENGES 167 Image, W. E., " A Case of Enlargement of the Left Mamma. To which is added an Anatomical and Pathological Description of the Tumours by T. G. Hake, M.D., and VV. E. Image. Communicated by Robert Liston, 'F.B..S."—Med.-CMr. Trans., 1817, xxx. 105. Eidd, Percy, "Cavernous Angioma of the Larynx." — Trans. Clin. Sue. Lond., 1892, XXV. 307. Lane, W. A., "A Case of Extensive Nsevus of the Peritoneum." — Trans. Clin. Soc, 1893, xxvi. 5. de Morgan, Campbell, " Remarks on some Cases of Vascular Tumour seated in Muscle." — Brit, and -For. Med. and CMr. Bev., 1864, xxxiii. 187. MtlUer, H., " Ein Fall von arteriellem Rankenangiom des Kopfes." — Beit. z. Idin. CMr., 1892, viii. 79. Power, D'Arcy, "Angioma of the Cerebral Membranes." — Trans. Path. Soc., 1888, xxxix. 4. Rau, F., " Gasuistische Mittheilungen von den Prosectur des Katharinenhos- pitals in Stuttgart (Cavernoses Angiom im rechten Herz-Vorhof)." — ■ Virchow's Arch., 1886, cliii. 22. CHAPTER XYI UTERINE FIBROIDS The tumours of the uterus known as fibroids, myomas, or fibro-myomas are extremely common, and on account of the difficulties and dangers which arise from them, directly and indirectly, their pathological and clinical aspects have been studied with very gi'eat care. Before minutely describing the structural peculiarities of fibroids, it will be of some advantage to study their topography and gross anatomy. Though fibroids arise in every part of the uterus, including its liga- ments, they are more common in the body of the organ than in its neck. Those which arise in the cervix offer peculiar features and demand separate consideration. Fibroids of the body of the uterus.— Tumours origin- ating in the uterine walls may be single or inultiple. In their early stages they resemble in section knots in a piece of wood. These tumours are firm, often hard, and always encapsuled. For clinical purposes it is convenient to divide them into three sets, according to the part of the uterus in which they arise : — 1. In the wall of the uterus : such are termed inter- stitial or intramural. 2. In the endometrium : these are said to be submucous. 3. In the layer of muscle-tissue subjacent to the peri- toneum : these are termed subserous. Fibroids may arise in and remain confined to any one of these situations, or all the varieties may be seen in the same uterus ; and there is no limit to their number. I have counted one hundred and forty fibroids in one uterus ; they varied in size from a dove's egg to that of a duck. It not infrequently happens that when a fibroid is confined 168 VTEBtNE FIBROIDS 169 to one wall ol the uterus and appears as a single tumour externally, it will be found on section to consist of two or more tumours growing in association, but eacb possessing its own capsule. Such may be conveniently called conglomerate fibroids. 1. Interstitial fibroids. — This variety may occur singly or in multiple. Such tumours in their early stages resemble, in Fig. 89. — Uterus in sagittal section showing interstitial and submucous fibroids. section, knots in wood ; they have distinct capsules, and are firm and even hard to the touch. The bundles of spindle- celled tissues are usually interwoven in such a manner as to present a very characteristic whorled appearance. There is no limit to their growth, and they sometimes attain a large size, and may weigh upwards of twenty and even thirty kilogrammes. 2. Submucous fibroids. — These tumours arise in the deeper parts of the endometrium, and, when they attain an appreciable size, project into the cavity of the uterus and 170 CONNECTIVE-TISSUE TUMOURS give rise to one variety of "fleshy polypus of the womb." Submucous tibro-myomas are at first sessile and invested on that surface which projects into the cavity of the uterus with mucous membrane. As they increase in size they dilate the uterine cavity and tend to become pedunculated. Fig. 90. — Very vascular fibroid in section. {After Virchoiv.) The presence of the tumour within the uterus leads to great thickening of the walls, accompanied by increased vas- cularity, which is often manifested by irregular haemorrhage from the uterus, or at least by profuse menstruation. Sub- mucous fibroids are sometimes so vascular as to resemble a cavernous nsevus on section (Fig. 90). The pedicle of a submucous fibroid may become sufficiently elongated to allow the tumour to pass through the cervical canal into the vagina, and even protrude at the vulva. When this happens an interesting change takes place in the epithelium UTERINE FIBROIDS m of the protruded part. So long as the tumour is contained within the cavity of the uterus, the epitheUum covering it is indistinguishable from that lining the cavity of the uterus. When the tumour enters the vagina, the columnar epithelium stratifies on the protruded surface, but that lining the glandular recesses remains columnar and retains its cilia (Fig. 91). 3. Subserous fibroids. — These arise from the uterine tissues g|T-ii^ir Fig. 91.— Microscopic characters of the ej^ithelium covering the protruded portions of a submucous fibroid ; it shows the mutation of columnar ciliated into stratified epithelium as a result of pressure. (Gervis.) subjacent to the peritoneal covering. When numerous they rarely attain large proportions. When the number is limited to three or four, one or more of them may attain moderate proportions ; like the submucous variety, they often become pedunculated, and when numerous they cause the uterus to assume a characteristic tuberous appearance. Sometimes as many as fifteen or twenty of these protuberances may be counted on a uterus, and they vary in size from a pea to a large walnut. In such cases, even when no intramural tu- mours are present, the walls of the uterus are thicker than natural. Subserous fibroids of this character rarely cause any 172 G0NNEGTIVE-TI8SUE TUMOURS inconvenience, and are often found after death in individuals in whom they have never j^roduced the least inconvenience during life, or in whom their presence has not been even suspected. The largest subserous fibroid I have removed weighed sixteen kilogrammes. In some rare instances the endometrium may be thickly beset with small fibroids varying in size from a mustard-seed to a dove's egg, the tumours being entirely confined to the tissues of the endometrium. I have seen three examples of this variety of the disease, and in each the number of fibroids exceeded one hundred ; in one of them several of the larger fibroids projected into the cavity of the uterus, and by mutual compression facets had been produced on their surfaces, so that on section of the organ, after hardening, the cut surfaces of the fibroids occupying the uterine cavity resembled in out- line a section through the bones of the carpus. In each instance the patients suffered from long-continued, profuse, and exhausting metrorrhagia. Latent fibroids. — If a number of uteri be examined, from women between the twenty-fifth and fiftieth years, by the simple means of sectioning them with a knife, in a large proportion of them numerous small rounded fibroids resembling knots in wood will appear, their whiteness being in strong contrast with the redness of the surrounding muscle-tissue ; these discrete bodies, in many instances no larger than mustard- seeds, are in histologic structure identical with the fully-grown tumours. A uterus may contain ten or more of them without the least distortion of contour or alteration in its size. These seedling fibroids may never cause trouble, may never pass beyond this stage, and often calcify in old age, but they may at any time grow and become formidable tumours. A careful consideration of the great frequency of seedling fibroids, and their multiplicity when compared with the num- ber of fibroids which attain a size sufficient to render them chnically appreciable, makes it undeniable that a large pro- portion of them remain latent. They may be compared to latent buds in trees (knors) and plants, on the ground that they may remain quiescent for a number of years and then assume active growth without any known cause. Latent fibroids have an important practical bearing ; it is CEBVIOAL FIBB0ID8 173 not an uncommon experience for an operator to dilate the uterine canal and abstract two or more submucous fibroids. However carefully the procedure may be conducted, and how- ever thoroughly the walls of the cavity may be examined for minute fibroids, no honest assurance can be given to the patient that other fibroids will not grow. Fibroids of the neck of the uterus. — These tumours do not arise so frequently in the neck as in the body of the Fallopian tu^he. Round ligament. Os uteri. Fig. 92.— An intracervical fibroid from a sterile married woman 40 years of age. The fundus of the uterus reached the level of the umbilicus. Uterus, but they are fairly frequent, sometimes attain large proportions, and possess peculiar features (Fig. 92). In the early stages of growth, cervical, like the common forms of uterine, fibroids are more or less globular, but when they exceed this size they tend to become ovoid. Fibroids may grow from any part of the cervix ; commonly they arise from its walls in such a way as to occupy the cervical canal (Fig. 93). These are known as intracervical or submucous 174 CONNECTIVE-TISSUE TUMOURS cervical fibroids. Less frequently they grow from the periphery of the cervix and do not invade the canal, but burrow under the peritoneum on the anterior or the posterior aspect of the uterus (Figs. 94 and 95). These are known as subserous cervical fibroids. The oval character of the cervical fibroid is best dis- played in the submucous variety, for as it grows it pushes the body of the uterus, which is perched on its upper pole, high into the abdomen, and in the case of very large tumours the fundus of the uterus can be detected as high as the navel. The topography and shape of this kind of tumour are best dis- played when the parts are sec- tioned in a sagittal direction. The oval shape of cervix fibroids is determined by the osseous boundaries of the true pelvis. In a normal female pelvis the pelvic diameter at the level of the middle of the cervix mea- sures, with the soft parts in posi- tion, about 10 cm. (4 inches); thus the lower segment of a large cervix fibroid is a solid cast of the true pelvis. In one of my specimens the minor (transverse) axis of the tumour measured 12 '5 cm., this ex- cessive measurement being due to the slow- but steady- expanding effects of the tumour on the bony walls of the pelvis. It is well to bear in mind that the oval condition of the vaginal p)ole of a large cervix fibroid corresponds with the shape of the occiput of a recently delivered foetus at term. The oval shape is also attained by subserous cervical fibroids when they grow from the posterior aspect of the cervix (Fig, 94). This kind of tumour as it increases in size pushes the body of the uterus high out of the pelvis on its upper pole, but its relation to the cervical canal is worth some attention. The intracervical fibroid (Fig. 93) Fig. 93.— Diagram to show the re- lation of an intracervical fibroid to the cervical canal. CERVICAL FIBROIDS 175 uniformly expands the cervix, and in very large specimens its tissues form a thin covering to the tumour ; but a fibroid of the posterior aspect of the cervix elongates it without expanding the canal, and is really situated between the cervix and the peritoneum. This is a topographical distinction of some importance in connexion with the clinical aspect of these tumours. Fibroids on the anterior aspect of the neck of the uterus remain more or less globular, and do not distort the shape of the cervix as a rule; when of large dimensions they push their way upwards between the peritoneum and the an- terior abdominal wall, and may roach as high as the umbilicus. It is a noteworthy feature of the cervical fibroid that in more than two-thirds of the cases the tumour is solitary. All varieties of cervix fibroids are furnished with a distinct capsule ; the tumour tissue on section presents the character- istic whorled arrangement of the common form of uterine fibroid, and is microscopically identical with it. Fibroids of the neck of the uterus Avhen they do not cause monorrhagia are very insidious, and rarely give rise to serious symptoms until large enough to fill the pelvis and to exert pressure on the urethra, the vesical segments of the ureters, and the rectum. In some cases, especially when the tumour is con- nected with the anterior aspect of the cervix, there is direct pressure on the bladder. The frequency of micturition, dysuria, and retention of urine, which are such common con- comitants of all varieties of cervix fibroids, are due to the bladder being dragged upwards by the uterus as this organ is pushed out of the pelvis by the growing tumour. In one example under my care, a woman 32 years of age Fig. 94. — Diagram of a fibroid growing from the posterior wall of the cer- vix, showing its relation to the peritoneum. 176 GONNEGTIVE-TISSTJE TUMOURS sought relief on account of a cervix fibroid which filled the vagina and prevented coitus ; it was successfully enucleated by the vaginal route. The largest intracervical fibroid known to me is a specimen (Hunterian) preserved in the museum of the Royal College of Surgeons of England, It measures 20 cm. in length and 12-5 cm. in width. Unfortunately it is without history. The largest cervix fibroid I have removed (Fig. 96) weighed 7 lbs. Fibroids of the mesometrium (broad ligament). — The connective tissue of the meso- metrium contains a quantity of plain muscle-tissue which is continuous with that directly underlying the peritoneal in- vestment of the uterus. This muscle-tissue is occasionally the source of tumours identical in structure with uterine fibroids. In the early stages these tu- mours are ovoid, encapsuled, and often bilateral ; they do not cause much inconvenience until they attain the size of coco- nuts ; even then they can be easily enucleated. They some- times grow with great rapidity, and in a few months form tumours weighing as much as ten kilogrammes, and, rising out of the pelvis, carry the uterus and its appendages with them. Some of the large globular tumours of the mesometrium are spindle-celled sarcomas (see p. 57). Doran has described some interesting cases and collected the literature, and he points out that they have been observed as early as the twentieth year. The majority occur, according to my ob- servation, after the thirty- fifth year. They are formidable tumours to deal with, but fortunately they enucleate easily. The largest specimen under my own care weighed thirteen kilogrammes and was successfully enucleated. Fig. 95.— Diagi-am of a fibroid growing from the anterior wall of tlie cervix, showing its rela- tion to the peritoneum as it passes from the anterior wall of the uterus to the bladder. FIBROIDS 111 Fibroids of the round ligament of the uterus.— This structure, like the ovarian ligament, is practically a process of the muscular tissue of the uterus, and tumours in all respects like the fibro-myomas of the uterus arise in this ligament, Fig. 96. — An intracervical fibroid in sagittal section. not only in the segment which lies in relation with the anterior layer of the mesometrium, but also in the terminal portion which traverses the inguinal canal. Fibroids of the ovarian ligament. — It is no uncommon M 178 CONNECTIVE -TISSUE TUMOURS thing to find a fibroid the size of a cherry in the ovarian ligament when the uterus itself is occupied by a crowd of fibroids; otherwise it is very rare to find a tumour in this process of the uterus, and especially one large enough to be obvious on clinical examination. Fibroids of the utero-sacral ligament. — Occasionally a fibroid is found burrowing under the posterior layer of the Fig. 97.— The cornua of a bicornate uterus in section ; each cornu contains an interstitial fibroid. Re- moved by subtotal hysterectomy from a woman aged 32 years, mesometrium and simulating a primary tumour of this structure, but when enucleated it is found attached to the side of the cervix near its junction with the body of the uterus by a very narrow, tendon-like stalk. It is probable that such a fibroid arises in the tissue of the utero-sacral ligament. In two examples under my own care the fibroids had a diameter of 12 cm. Fibroids in malformed uteri. — Fibroids not only grow from uteri of normal shape, but they have been observed in double uteri of various kinds (Fig. 97), and even growing from the rudimentary cornu of the so-called " unicorn uterus " (Doran, Bland-Sutton, and Routh). A double uterus is liable to be the seat of any kind of tumour which attacks a uterus of normal shape ; but a fibroid growing in a malformed uterus of any kind is a rare occurrence. REFERENCES 179 Bland-Sutton, J., "A Tumour of the Mesometriutn weighing twenty-two pounds."— rra?i.s. Obstet. Soc, 1899-1900, xli. 298. Bland-Sutton, J., " On some Cases illustrating the Surgery of the Uterus." — Clin. Journ., 1901-2, xix. 1 (" On Fibroids in a Unicorn Uterus," p. 8). Doran, A., " Fibroid of the Broad Ligament, weighing forty-four and a half pounds (twenty kilogrammes), removed by Enucleation ; Eecovery. With Table and Analysis of Thirty-nine Cases." — Trams. Oistet. Soc, 1899-1900, xli. 173. Doran, A., " The Kemoval of a Fibroid from a Uterus Unicornis in a Parous Subject."— ^rii!. Med. Juurn., 1899, p. 1389. Routh, Amand, "Fibroid of One-Horned Uterus." — Trans. Oistet. Soc, 1887-88, xxix. 2. CHAPTER XVII STRUCTURE AND SECONDARY CHANGES OF UTERINE FIBROIDS Uterine fibroids differ miicli in texture : some are as liard as cartilage, and a few, when calcified, resemble porous stone ; others are as soft and succulent as a ripe orange, and occasionally some are like jelly. Between these extremes every degree of hardness or softness occurs ; but they all agree in one particular — namely, in the possession of a well-developed capsule, a structure of vital importance to a fibroid, as its life depends upon it. Hard fibroids are yellowish-white on section, softer specimens resemble the normal colour of the uterus. Soft tumours, as a rule, grow quickly, and are very vascular, but the hardest and the gelatinous fibroids are poorly supplied with blood. It is by no means uncommon to find a uterus possess- ing many fibroids (twenty or more), some of which are very hard ; one or more may be calcified, others are of the same density as the wall of the uterus, while one or more are soft and even diffluent. Attention has already been drawn to the fact that the only structural feature fibroids have in common is a well-marked capsule, of fibrous tissue, which completely isolates the tumour proper from the uterine tissue. Even in com- pletely calcified fibroids a thin capsule can be demonstrated, and occasionally the only solid representative of the fibroid is the capsule, the originally solid parts of the tumour having slowly liquefied (Fig. 98). Fibroids changed in this way are often referred to as " fibre- C3^stic " tumours. In some instances the capsule of a fibroid calcifies and encloses the tumour in a more or less complete shell. Fibroids in this condition are dead, and on section exhibit the dirty yellow colour of chamois leather, and equal it in toughness. 180 UTERINE FIBROIDS 181 The most typical variety of " fibroid " not only resembles the wall of the normal uterus in toughness, but is similar to it in microscopic structure, and consists of unstripecl muscle-tissue, which has a remarkable tendency to be arranged in whorls (Fig. 99). The very hard fibroids are composed of tissue which Fig. 98. — A sessile subserous fibroid which had undergone extensive mucoid degeneration. From a sterile married woman 37 years of age. microscopically resembles dense fibrous tissue, Avith here and there strands of cells resembling unstriped muscle cells. This variety is often called fibro-myomas, and its mem- bers display the whorled arrangement in a very striking manner. The fibro-myomas are very liable to calcify. The deposit of earthy salts does not take place in an irregular manner, but follows the disposition of the fibres, and the 182 CONNECTIVE-TISSUE TUMOUES whorled arrangement is seen when the sawn surface is examined (Fig. 100). When incompletely calcified tnmom's are macerated, and the decayed tissue is washed away, the cal- careous matter remains as a coherent skeleton of the tumour. Such changes have taken place whilst the tumour remained in the living uterus; they were formerly termed "womb stones." OccasioDally, in old women the uterus attempts to extrude a calcified fibroid; when the tumour is large the Fig. 99. — Minute structure of a young uterine fibroid ; the circular cells are spindle cells cut at right angles. This figure represents a complete section through the equator of a seedling fibroid the size of a mustard-seed. result, if left to the efforts of nature, is as a rule disastrous. The extraction of such a tumour by art is difficult and tedious. When "calcified fibroids" have been found in coffins, in old burying-grounds, they have been mistaken for vesical calculi. UTERINE FIBROIDS 183 The soft, jelly-like fibroids are, in the majority of cases, due to secondary (myxomatous) changes in tumours which were originally hard. This is proved by the fact that patches of softening are found in hard tumours, and occasionally fibroids come to hand in which the very hard, calcified, gelatinous and difiluent tissues co-exist. However, it is important to remember that these changes do not always depend on the age of the tumour, for a very large proportion of uterine fibroids which occur before the thirtieth year are myxomat- ous. What is more important, these soft (almost liquid) fibroids are locally malignant — that is, they recur if enu- cleated, and this sometimes happens very quickly. In Fig. 100. — Calcified uterine fibroid in section. (3Iuseicm, 3fiddlesex Hospital.) 1898 I removed through the vagina a jelly-like fibroid, as big as an orange, from the uterus of a woman 45 years of age, and was careful to remove the whole capsule. In six months she returned with a tumour in the uterus occupying the position of the original fibroid, but twice its size. Hysterectomy Avas performed, and the uterus con- tained a large myxomatous fibroid. She remains free from recurrence. Women with hard fibroids rarely complain of them, but when the fibroid is soft like jelly the health of the patient is markedly impaired, quite apart from the anaemia due to monorrhagia. Red degeneration (necrobiosis). — This change in fibroids 184 GONNEGTIVE -TISSUE TUMOURS is best studied in specimens which are complicated by pregnane}^ It is considered at p. 199. Malignant changes in fibroids. — It is believed by many that a sarcomatous change may arise in uterine fibroids. The matter has been carefully considered by competent men, and a critical examination of the evidence makes it clear that in a very large proportion of the cases described as " sarcomatous degeneration of a fibroid" the changes were due to septic infection. In all future records published as evidence in this direction there must be a careful account of the minute structure of the tumour by a competent pathologist. The great defect in nearly all the recorded cases in which the malignant change has been suspected is the absence of any description of the mode of death where the patient survived the operation. Sarcomas are so prone to disseminate, that any patient who has died in consequence of malignant degeneration of a fibroid would be expected to have secondary nodules in the lungs at least. The most convincing case which has come under my notice occurred in a woman 59 years of age : she died in the Middlesex Hospital under the care of Dr. Finlay. I made the post-mortem examination. The uterus contained a fibroid as big as a child's head, attached to the fundus of the uterus : it was adherent to and had penetrated the bladder and intestine. Secondary nodules were found at the base of the right lung, on the wall of the left cardiac ventricle, and in the left kidney. The microscopic characters of the uterus were those of a myoma and a spindle-celled sarcoma. The secondary nodules displayed the same structure. Griffith and Williamson have recorded in detail the case of a woman aged 56 who died in St. Bartholomew's Hospital with a sarcomatous fibroid. The uterus contained several fibroids, and secondary nodules were found in the lungs. In many cases reported as fibroids undergoing malignant change the tumours were in all probability sarcomatous from the beginning. They should be called sarcomatous fibroids. An attempt has been made by Piquand to formulate the symptoms and diagnostic features of sarcomatous disease of the uterus. He attempts to arrange the disease under three headings, thus : sarcoma of the interstitial tissue of the UTERINE FIBROIDS 185 uterus ; sarcoma primary in the endometrium ; and sarcoma of the neck of the uterus. Of these three groups, that of the racemose sarcomas of the neck of the uterus is the most distinctive and easiest of recogni- tion, chnically and microscopically (see p. 56). In regard to sarcoma of the body of the uterus and the endometrium there is great difficulty. As a matter of fact, there is every gradation from the hard fibroid and the typical myoma to the soft, Fig. 101. — The body of the uterus in coronal section, showing a large fibroid traversed by narrow tortuous canals — probably lymph-spaces. diffluent myxoma. Among the softer forms we meet with the spindle-cell sarcoma (the recurrent fibroid of older writers), which surgeons fail to recognize until the patient comes under observation with signs of local recurrence. In this particular histology fails us, in spite of its triumphs. Fibroids complicated with cancer of the uterus.— This sinister combination is discussed in Chap, xxxviii. Lymphatics in fibroids. — It is not uncommon, when removing large uterine fibroids by cceliotomy, to find lymph- vessels in the broad ligaments as big as the radial, or even 186 CONNECTIVE-TISSUE TUMOURS of the size of ttie axillary A^ein. Occasionally a firm fibroid will present on section numerous irregular tortuous channels (Fig. 101). These are probably lymph-spaces. It has hap- pened to me on several occasions, when operating on sub- serous fibroids, to find the tumour adherent to the great omentum, and the arteries, veins, and lymphatics in the adherent portions of the omentum were so enormously developed as to form a mixed rete mirabile ; the arteries bemg in many instances as big as radials, the veins equal to the cephahc, and the lymphatics of the size of goose-quills. The contrast of the maroon tint of the arteries, the deep blue of the veins, and the light yellow of the thin-walled lym- phatics formed an anatomical picture scarcely likely to be forgotten by one who has had to deal with such a condition. Rate of growth of fibroids. — On this subject there are very few facts forthcoming. In general terms it may be stated that soft fibroids grow quickly, the hard ones increase very slowly; those fibroids grow most quickly which soften, and it is a remarkable fact that when the myxomatous change is established in one of these tumours it will often increase in size with astonishing rapidity. The only observation I have been able to make with any accuracy in regard to the rate of growth of fibroids is the following : — In 1896 I enucleated by means of an abdominal incision from the uterus of a woman 23 years of age (I obtained a copy of her birth certificate) a fibroid measuring 15 cm. in its major and 5 cm. in its minor axis. The patient, already mother of one child, was delivered of a healthy baby eight months after the operation : it was reasonable to believe that she had become pregnant immediately before coming into the hospital. Three years later (1899) this woman again came under my care on account of a pelvic tumour : this was watched for three months, and it increased so much that it became necessary to perform hysterectomy. The uterus contained twenty tumours varying in size from a ripe currant to a hen's Qg^. The largest tumour occupied the cervix. There were no signs of these tumours when the patient was sub- mitted to myomectomy in June, 1896. CHAPTER XVIII MODES IN WHICH UTERINE FIBROIDS IMPERIL LIFE It is too true that fibroids are the commonest of all the species of tumours to which women, whether married or single, fruitful or barren, are liable. It is also a fact that the uterus may contain one fibroid or many and cause neither inconvenience nor sufferhig — indeed, the individual owning them is ignorant of the existence of tumours in her womb ; but it is equally true that they are often the source of much suffering, and occasionally cause death in insidious ways, some of which will be considered. Haemorrhage. — This is the commonest of all the incon- veniences which fibroids cause, but it is confined to those which implicate the endometrium. The bleeding occurs under two conditions ; most commonly it takes the form of excessive loss at the normal menstrual periods (menorrhagia). The most serious haemorrhages are associated with septic changes in the tumour. It is a fact of some importance that a small submucous fibroid will induce such profuse bleedings at the menstrual period as to place life in immi- nent peril ; whilst a Jarge interstitial tumour, even though it project into the uterine cavity, scarcely influences the loss. When a woman with a fibroid bleeds excessively between as well as at the normal menstrual periods, it often indicates that the tumour has become septic. It is important to realize that oft- repeated losses of blood continued over a long period not only lead to profound anaemia, but also to grave changes in the heart-muscle, which frequently end in sudden death, as well as greatly adding to the operative risks when such individuals submit to surgical procedures. These changes have been carefully described by Wilson. 187 188 GONNEGTIVE-TISSUE TUMOURS Septic infection. — This is, perhaps, the most serious complication of a fibroid, and even when it does not cause death is always attended with dangerous consequences. In- fection of a fibroid may arise in a variety of ways — e.g. the extrusion of a submucous tumour into the vagina exposes it to injur}^, and micro-organisms gain access to it through abrasions in its capsule. Infection may be due to injury from the uterine sound or dirty dilators, or septic changes super- vening on labour or miscarriage ; occasionally it is due to intestinal gases when bowel adheres to the tumour. An in- fected fibroid is a soft, dark-coloured, stinking mass, which Fig. 102. — Body of the uterus in section showing two sessile submucous fibroids. The capsule of one has ulcerated and the tumour become gangrenous. The patient suffered from excessive and almost continuous bleeding. bleeds freely when touched. In the early stages of the infection it appears on section oedematous, and exhales a sickly odour. On microscopic examination the muscle-cells are separated by multitudes of inflammatory cells, and colonies of pathogenic micro-organisms can by special methods be demonstrated among the inflammatory cells. When a large fibroid becomes septic it gives rise to severe constitutional disturbances (sejDticiemia), like gangrene of other organs, and will, unless promptly removed, inevitably destroy Hfe (Fig. 102). Small fibroids when septic, though they give rise to serious trouble, do not so urgently threaten life, but they work great mischief, for the infection is sure to involve the adjacent UTERINE FIBROIDS 189 endometrium (which sometimes sloughs) and creep into the Fallopian tubes. The septic matter in some cases becomes imprisoned in the tubes by occlusion of the coelomic ostium ; this is a fortunate event. Occasionally it leaks directly into Fig. 103. — Section of a uterus from which a sloughing fibroid had been removed. The mucous membrane was gangrenous, and infective material had leaked into the coelom through the unclosed ostium. the peritoneal cavity through an unclosed ostium, and estab- lishes fatal peritonitis (Fig. 103). An extruded fibroid often becomes septic, for , when the tumour passes beyond the external orifice of the uterus, 190 GONNEGTIVE-TISSUE TUMOURS the part lying within the canal is firmly grasped by the ute- rine walls bounding the internal orifice. Should the tumour be very vascular, the venous circulation is interfered with, and the projecting part becomes cedematous. Should the com- pression continue, the extruded parts become congested, and may even necrose, and as the dead tissue is in a situation easily accessible to air, and consequently to putrefactive organisms, gangrene, with all its attendant evils, is the result. It is always necessary, in examining fibroids projecting into Fig. 104:. — Partial inversion of a uterus due to a fibroid. the vagina, to be careful to distinguish between the fundus of an inverted uterus and a fibroid extruded from the uterus, and at the same time to remember that a submucous fibroid will occasionally invert the uterus (Fig. 104). Sepsis plays an important part in haemorrhage associated with cervical fibroids. Professional opinions on this matter are very divergent, and after a careful study I am able to state that menorrhagia and metrorrhagia are only associated with the intracervical variety of fibroids and bear no relation to the size of the tumour; but hsemorrhages only occur with the intracervical fibroids when the uterus has made attempts to extrude, or has succeeded in extruding, the tumour wholly UTERINE FIBROIDS 191 or partially into the vagina. The corollary is obvious. An extruded or partially extruded fibroid quickly becomes septic, and as surely as this happens menorrhagia and metrorrhagia Myoma Bladdei Eectum. Urethra. Vagiua. Cervix. Fig. 105. — Frozen section of a pelvis containing an impacted uterine fibroid. are unfailing consequences, whether the fibroid be large or small. When the orifice"of the cervical canal remains a "mere dimple," menstruation is normal, and the patient is usually a spinster, or, if married, barren. 192 CONNECTIVE -'TIS SUE TUMOURS Impaction and its effects. — A fibroid is said to be im- pacted (or incarcerated) when it fits the true pelvis so tightly that the tumour cannot rise upwards into the belly. All varieties of fibroids may become impacted, and, as the complication is of great clinical imj^ortance, it needs detailed consideration. A subserous fibroid growing from the fundus will often produce retroversion of the uterus, and the tumour occupies the hollow of the sacrum. As the tumour grows it appro- priates the available pelvic space, and in due course exerts pressure on the rectum and urethra, interfering with defse- cation and micturition (Fig. 105). A solitary intramural fibroid may be small enough to rest in the true pelvis without pressing unduly on the urethra or ureters. Presently it increases to such a point that the turgescence which precedes the menstrual flow will cause it to press the urethra against the symphysis, and set up reten- tion of urine. When menstruation occurs the turgidity of the tumour subsides, and the urethra is set free. Frequent recurrence of this pressure permanently damages the bladder and kidneys. Very vascular tumours yield a loud murmur or hum on auscultation, a sign of very great value in differential diagnosis. In many cases I have been able to demonstrate the existence of a loud murmur for a few days before menstruation, but it disappeared with the flow of blood, and remained in abeyance until a few days before the succeeding period. The most insidious, and therefore the most dangerous, variety of impaction is that which occurs with cervix fibroids. It has already been mentioned that when one of these tumours attains a transverse diameter of 10 cm. (4 inches) it has practically used up the spare pelvic space and exerts injurious pressure on the rectum, ureters, urethra, or bladder. Most commonly it j^resses on the neck of the bladder and causes retention, leading to frequent and pain- ful micturition. It is one of the most striking features of the cervical fibroids that they rarely cause bleeding except when they extrude from the mouth of the uterus and become infected, and only cause inconvenience when they interfere with the bladder. Herein lies the danger, as grave tJTEBINE FiBEOIDS 193 injury is often wrought on the pelvis of one or both kidneys before the existence of the tumour is even so much as suspected. It is an important fact to remember that token a woman between 35 and 45 years of age seeks relief because she suffers from retention of urine for a few clays 'preceding each menstrual 'period, it is cdrtibst certain that she has a fibroid in her uterus. Axial rotation. — The method by which fibroids under certain conditions accommodate themselves in the pelvis is worth further note. When one growing fibroid occupies the posterior wall of the uterus and another its anterior, so long as the total antero-posterior diameter of the uterus with its tumours does not exceed 10 cm. it may occupy a normal position. When this diameter increases, the uterus slowly rotates, and the larger tumour will occupy the trans- verse diameter of the pelvis. If growth continues, it gradually fills up the available pelvic space^, and impaction slowly but surely ensues. As a rule the tumour in the posterior wall lies in the recto-vaginal fossa, but occasionally the uterus will be so rotated that the tumour in the anterior wall occupies the space in the true pelvis, and that in the posterior wall pro- jects into the hypogastrium. A subserous fibroid with a long and slender stalk is liable to rotate and twist its pedicle, a movement which causes very great pain. Some small calcified pedunculated fibroids may be so twisted that they become detached. A loose body of this kind has been found in the sac of an inguinal hernia. Intestinal obstruction. — Uterine fibroids may obstruct the intestines in three ways, thus : — 1. A pedunculated subserous fibro-myoma, especially if its stalk be long and narrow, may entangle a loop of small intestine and lead to fatal obstruction. This may happen with small as well as Avith large tumours. 2. A very large fibroid rising high in the abdomen may rest upon the pelvic brim in such a way as to obstruct the sigmoid flexure. 3. An impacted fibroid may press upon the rectum and lead to obstinate constipation and chronic obstruction, with all its inconveniences and evils. Apart fi'om the various modes already mentioned in which fibroids cause death, they may destroy life in unexpected N 194 CONNECTIVE-TISSUE TTJMOUTiS ways, of whidi the following is a remarkable and very unusual example recorded hy Arnott : A maiden lady of 72 years was knocked down by a large dog and fell forwards on the pavement. She was seized with severe pain in the belly, and died in thirty-four hours. At the autopsy a circular orifice was found in a coil of ileum which lay between the anterior abdominal wall and a calcified tumour of the uterus. There was extravasation of fseces and intense peritonitis. The tumour, which was as large as a child's head, apparently originated in the anterior wall of the uterus. Several small tumours, also calcified, were attached by pedicles to its capsule. A spinster aged 43 fell heavil}' on an asphalted walk, and felt severe pain in the belly. Some hours later coeli- otomy was performed, and a large pedunculated fibroid weighing 6| lb. removed. A vein on its surface had been lacerated by the fall and had bled fi-eel}^. She recovered. (Littler.) Arnott, James M., " Case of Large Osseous Tumour of the Uterus " — JHed.- Cldr. Tram., 1840, xxiii. 199. Bland-Sutton, J., "A Uterus showing the Effects of a Gangrenous Fibroid." — Tram. Ofjstet. Soc, 1890-91, xxxii. 171. Bland-Sutton, J., "Acute Axial Rotation of a Calcified Pibroid of the Uterus. —Trans.. Obstet. Soc, 1904, xlvi. 149. Wilson, T., " The Relations of Organic Affections of the Heart to Fibro-Mvoma of the Uterus." — Trans. Obstet. Soc, 1900, xlii. 176. CHAPTER XIX RELATION OF UTERINE FIBROIDS TO MENS- TRUATION, CONCEPTION, PREGNANCY, PUERPERY, AND THE MENOPAUSE There is nothing in oncology better established than the fact that all uterine fibroids arise during the menstrual period of life. In Great Britain this period has an average of thirty years, from the fifteenth to the forty-fifth year. There are, however, few reliable records of fibroids being found in the uterus before the twentieth year. Submucous fibroids have been removed by coeliotomy from girls of 18 years (Scharlieb and Madden). Many examples have been observed between the twentieth and the twenty-fifth years. Between 25 and 35, fibroids are fairly common, but the maximum frequency is attained between the thirty-fifth and the forty-fifth years. The interval between the twenty-fifth and thirty-fifth years is the great child-bearing period, with an average length of twelve years. The menstrual epoch of a woman's life, in relation to pregnancy and fibroids, may be divided into three periods, thus : — 1. From 15 to 25, in which, assuming the environment to be favourable, a woman is infinitely more Hable to con- ceive than to grow a fibroid. 2. From 25 to 35 ; during this period her liability to pregnancy is greater than in the preceding period, but her liability to fibroids is also greater. 3. From 35 to 45 ; in this the liability to conception is greatly diminished, but the liabihty to fibroids is immensely increased. Not only is it true that fibroids arise during menstrual hfe, but it is equally certain that they influence menstruation, and I have operated on many cases in which this disagreeable 195 196 OONNEGTWE- TISSUE TUMOURS phenomenon has been as profuse between 50 and 55, and even at 60, as it was at 20. It is questionable whether the fluxes of blood in women wdth uterine fibroids after the age of 50 years should be regarded as menstruation in the proper acceptation of the term. Fig. 106. — Pregnant nterus with multiple fibroids; removed by operation. After the uterus bad been removed, an incision was made inthe uterine wall, and, as rigor mortis supervened in tbe organ, tbe embryo iu its amnion was extruded. If the conclusion is correct that the interval from 25 to 35 is the great child-bearing period of a woman's life, it foUows as a corollary to the three deductions in the pre- ceding section that, when pregnancy and fibroids co-exist, the subjects of such a combination should be women past 30, and these should, as a rule, be those who have either FIBROIDS AND MENSTRUATION 197 married late in life, or, if niarried early, have remained for many years sterile. It is universally admitted by writers who have devoted careful attention to the matter that the presence in the uterus of a submucous or of a large interstitial fibroid is very unfavourable to conception. A fibroid of either variety, or one in the neck of the uterus, is by no means a bar to conception, or even to successful pregnancy, but such a combination is very dangerous to the mother Fig. 107. — Myomatous gravid uterus in sagittal section. At the beginning of the third month imjiaction occurred ; this was relieved, and, as the uterus with its tumoiu'S was too long to lie in its natural position, axial rotation occurred. The antero-posterior length of the distorted organ was 20 cm. Only a portion of the large tumour is shown in the figure. and to the child. Two facts may be stated with a fair amount of accuracy thus : 1. When the uterus of a parous woman begins to grow a fibroid, she usually ceases to conceive. 2. When a woman whose uterus contains a fibroid con- ceives, this event is usually preceded by a long period of unfruitful wedlock. A large subserous fibroid does not in- fluence conception, but is occasionally a serious complication of pregnancy as well as of delivery and puerpery. 198 CONNECTIVE-TISSUE TUMOURS A large fibroid in the neck of the uterus hinders but does not prevent conception ; it is, however, a seri- ous obstacle to successful delivery, and it is admitted by all practitioners who have had experience in mid- wifery that the most serious obstruction which arises in connexion with uterine fibroids is caused by a large tumour in the neck of the uterus. In such a case I performed Fig. 108. —Uterus in sagittal section; its neck is occupied by a large intracervical fibroid. There is also a submucous fibroid. (From a barren woman aged 41, who had been married many years.) total hysterectomy with success after labour had begun (Fig. 110). Rutherford Morison has had a similar experience. Inimicality of pregnancy and uterine fibroids. — The banefiilness or harmfulness of the association of pregnancy is of three kinds : 1. Obstructive. — The harm which may arise from the obstruction offered by a fibroid to a gravid uterus sometimes occurs early in the pregnancy because it may lead to impac- tion and even slow torsion of the uterus. If the fibroid be pedunculated the upward movement of the uterus may FIBROIDS AND PBEGNANCY ]99 cause it to rotate and twist the pedicle ; occasionally it will be incarcerated by the uterus. 2. Septic infection. — -An interstitial or a submucous fibroid may be infected from careless attention to antiseptic details following miscarriage or delivery at term : many puerperal Fig. 109. — Pregnant uterus clefoiined by fibroids ; the largest grew in the neck of the uterus. women have lost their lives from this cause. Occasionally a submucous fibroid may be extruded into the vagina during delivery, but this is rare. A subserous fibroid may become cedematous, and when the uterus expels the foetus the tumour may become septic and set up peritonitis, which may destroy the patient or lead to the formation of dangerous adhesions. 3. Degeneration of the fibroid. — This is an insidious 200 GONNEGTIVE-TISSTIE TUMOURS danger, and one which has not been fully appreciated by obstetricians, for it is a condition often associated with preg- Fig. 110. — Gravid uterus in sagittal section. The patient miscarried at the seventh month, and the arm presented. Delivery heing impossible on account of a large cervical fibroid, the uterus with its cervix was removed. The cedema of the presenting arm is well shown. {Museum of the RoyaJ CoUetje of Surt/eons.) nancy apart from septic infection, or from mechanical injury which the tumour may receive in the course of the gradual FIBROIDS AND PREGNANCY 201 enlargement of the uterus, or during its sudden diminution after delivery. Moreover, the change which pregnancy induces in fibroids has interested me for many years, and I have been able to collect a large number of facts from personal observation. rig. 111.— Gravid uterus deformed by fibroids which were soft and red, while one was difluent. Eenioved from a woman aged 28 on account of pain, impaction, and rotation of the uterus. The arrow lies in the cervical canal. The usual colour of a uterine fibroid is pale yellow; in many degenerating and necrotic fibroids this colour deepens. In the course of pregnancy a fibroid, especially one of the interstitial kind, assumes a deep-red or mahogany tint. In the early stages the tumour exhibits the colour in streaks, but as the pregnancy advances it permeates the whole tumour. Occasionally, even in the mid-period of pregnancy, this 202 GONNEGTIVE-TISSUE TUMOURS necrotic change may be so extreme that the central part of the tumour is reduced to a red pulp. In 1903 Fairbairn wrote an excellent j)aper on this necrotic change in fibroids, and it is now becoming familiar as the "red degeneration." Until Fairbairn began to accu- mulate the material for this paper I held the opinion that this change was only seen in association with pregnancy, Fig. 112. — Uterus distorted with, fibroids, and containing a foetus of four months' development. (From a woman aged 42 years.) but he soon convinced me that it occurred in- sjDinsters, and I have myself since seen well-marked examples in women who have never been pregnant. At the same time it must be stated that the largest number, the best marked, so far as colour goes, and the most extreme examples of this red degeneration occur in association with j)regnancy. In the earl}'- cases which came under my notice, the redness of the cut surface of these tumours so strikingly resembled beefsteak that it suggested to me, and appears to BED DEQENEBATION 203 have done so to other observers, that the change in colour might be due to an increase in the muscle-fibres in conse- quence of the physiological enlargement of the uterus. The microscope, however, dispelled this illusion, showing, the colouring material to be blood-pigment diffused through the necrotic tissue of the tumour. Murray ascribes the redness af these fibroids to necrobiosis accompanied by haemolysis and diffusion of the blood-pigment. Smith and Shaw succeeded in finding micro-organisms in these red degenerate fibroids. They are not present in all, Hastings isolated and obtained in pure culture Staphy- lococcus pyogenes aureus from one of my specimens, but as a rule these softened tumours are sterile. This red degeneration is of interest outside the pathological laboratory : it is of clinical importance, because fibroids which are undergoing this peculiar change are often painful and ex- tremely tender. This tenderness is a valuable diagnostic sign. Kefiections on the complications resulting from the pre- sence of fibroids in the walls of a pregnant uterus make it obvious to any thoughtful practitioner that this may be described as a malicious combination. Fibroids and tubal pregnancy. — This is a rare com- bination, but it occurs, and the co-existence of a gravid tube and large or moderate-sized fibroids in the uterus gives rise to unusual difficulty in diagnosis. How great this may be is set forth in a case described with great care and detail by Cullingworth in 1898. The literature of this unfortunate combination has been collected by Frank E. Taylor. It is very scanty. Fibroids and the menopause. — It was formerly taught and believed that uterine fibroids cease to be troublesome with the cessation of menstruation. It is quite certain that this opinion requires reconsideration. Uterine fibroids are peculiar in their age-distribution, for, as has already been mentioned, they only arise during menstrual life (15 to 45), but they stand absolutely alone among tumours in possessing another remarkable character : as a rule, they cease to grow after the menopause, and in some instances they undergo a marked diminution in size. To-day no gyna3Cologist seriously believes that uterine fibroids disappear. 204 G0NNEGTIVE-TI88UE TUMOURS Though fibroids, as a rule, cease to grow after the meno- pause, it must not be forgotten that they soiQietimes take on unusually rapid growth at this period ; and, apart from this, they ■ are often sources of great peril to life by co-existing with other serious diseases of the uterus, tubes, and ovaries ; while the very fact that they are apt to diminish in size is occasionally a source of danger. Apart, however, from these considerations, the fibroids are themselves sources of trouble on account of the degenerate and septic changes to which they are liable. It is also very essential to bear in mind that the existence of a fibroid in the uterus has in a very large propor- tion of cases a malicious influence in delaying the menopause. The uterus has often been removed from patients between 50 and 60 years of age in whom monthly fluxes of blood were as regular as, but much more profuse than, in women of 20 years. On the other hand, occasionally a woman may have her menopause at the forty-second or forty-fifth year, though a large fibroid is connected with the uterus. The fact that a fibroid may shrink after the menopause is in itself frequently a source of danger, especially when it is pedunculated, for the tumour may be so big that its size prevents it from tumbling into the pelvis, but after the shrinking consequent on the menopause such a fibroid may fall into the true pelvis and become impacted. It is an uncommon complication, but it happens. The most frequent and most dangerous alterations in fibroids after the menopause are necrotic and septic changes. During menstrual life fibroids generally enjoy an abundant blood-supply ; in some instances they are almost as vascular as naevi. On the occurrence of the menopause, the cessation of the menstruation is accompanied by a remarkable abate- ment in the blood-supply^ and not only does the tumour cease to grow, or even shrinks, but the very fact that its nutritive irrigation, so to speak, is arrested leads to degenerative changes, and the fibroid becomes in many instances a dead, sequestered body, which may calcify. So long as septic organisms are denied access it will remain inert ; but when, from various causes, putrefactive organisms gain access to it, the results are often dire in the extreme. It is far easier to prove that putrefactive organisms obtain FIBROIDS AND THE MENOPAUSE 205 access to dead or dying fibroids than to tell how they get to them. There is, however, one mode of access which is un- deniable. The fibroids which give rise to most trouble after the menopause are those of the submucous variety, and there seems a strong tendency, v^^hen the uterus passes into its resting stage and the fibroid is shrinking and dying, for the organ to attempt the extrusion of the tumour. A careful study of the cases which have come under my observation teaches me that a fair proportion of troublesome post-meno- pause fibroids have undergone partial extrusion, or the mouth of the womb being widely dilated facilitates the ingress of pathogenic micro-organisms. A study of the post-menopause behaviour of uterine fibroids and the perils they entail indicates in no uncertain manner that even in obsolescence they are often mischievous and insidiously lethal. The relation of submucous fibroids to cancer of the uterus is discussed in Chap, xxxviii. Bland-Sutton, J., " Essays on Hysterectomy," London, 1905. CuUingworth, C. J., "Early Ectopic Gestation (? Tubo-Uterine) complicated by Fibro-Myoma of the Uterus."-— Trans. Obstet. Soe., 1898, xl. Fairbairn, J. S., " A Contribution to the Study of One of the Varieties of Necrotic Change in Fibro-Myomata of the Uterus." — Jotirn. of Oistet. and Gyn. of Brit. Emp., 1903, iv. 119. Morison, Rutherford, Nortliumierland and Durham Med. Jonrn., July 190J:. Murray, H. L., "The Haeniolytic Lipoids of Degenerating Fibroids, with special reference to Eed Degeneration." — Journ. of Obstet. and Gyn., 1910, xvii. 534. Smith, J. L., and Shaw, W. F., " Pathology of the Eed Degeneration of '¥ihxo\ds."— Lancet, 1909, i. 242. Taylor, F. E., "Extra-Uterine Gestation associated with Fibro-Myomata." — Joimi. of Obstet. and Gyn. of Brit. Emp., 1906, ix. 412. CHAPTER XX CLINICAL CHARACTERS AND TREATMENT OF UTERINE FIBROIDS Clinical characters. — Uterine fibroids, though exceedingly common, are unknown before puberty, and rarely attract attention before the twentieth year; they are most common between the thirtieth and fiftieth years. In a large propor- tion of patients the earliest indication of the presence of a fibroid in the uterus is excessive menstruation (monorrhagia); this is often the only symptom which leads the patient to seek advice, and on examination a large pelvic tumour may be detected. In many cases there is no obvious enlargement of the uterus; the fibroid, though big enough to cause severe bleeding, is not large enough to be detected until the cavity of the uterus is explored through a dilated cervical canal. In many instances, when the patient seeks advice the tumour is actually presenting at the mouth of the womb. Fibroids large enough to rise out of the pelvis usually oc- cupy the hypogastrium, but when stalked they may lie laterally and simulate ovarian tumours. On palpation fibroids may be smooth, but when their surfaces are tuberous it is a valuable sign. Auscultation sometimes furnishes useful evidence, for soft, rapidly growing fibroids often yield a loud hum syn- chronous with the pulse, like the murmur heard during pregnancy. This venous hum is most frequently detected shortly before the onset of a menstrual period. On vaginal examination, the tumour may be found closely associated, and often incorporated, with the uterus. When the tumour occupies the cervix, the whole organ feels like a globular body, and the mouth of the womb is indicated by a mere dimple. The diagnosis of a fibroid in the uterus is often rendered difiicult by comj)lications such as pregnancy, uterine, tubal, or even cornual; the co-existence of ovarian 206 TREATMENT OF UTERINE FIBROIDS' 207 cysts and solid tumours; tubal conditions such as hydro- salpinx and pyosalpinx and primary cancer of the Fallopian tube ; tumours of the pelvic bones and connective tissue. When a woman has a tumour suspected to be a fibroid, and there is reason to believe that it is rapidly increasing, it is worth while to remember — 1. That she may have conceived, so that the enlarge- ment is due to the progress of the pregnancy. 2. The tumour may have become septic, or secondary changes 'may have led to the formation of cyst- like spaces. 3. The diagnosis may he erroneous, and the suspected fibroid may be really an ovarian tumour. 4. Ovarian tumours and uterine fibroids often co-exist. 5. An over-distended bladder has many times been mistaken for a rapidly growing pelvic tumour. 6. Hydrosalpinx, pyosalpinx, primary cancer of the tube, and even tubal pregnancy sometimes com- plicate fibroids. Even this list does not exhaust the possibilities, for a myomatous uterus may become impacted in consequence of conception, and, when the impaction is relieved, axial rota- tion may occur. Treatment. — All attempts to cure fibroids by drugs or by means of electricity have been conspicuous failures, so that patients whose lives and usefulness are threatened by these tumours are obliged to seek the aid of surgery. It is true that fibroids often occupy the uterus for years and cause no trouble, but many give rise to severe bleeding, and place life in great jeopardy. Kecurrent bleeding is the most common condition which leads women with fibroids to seek medical advice. Pelvic pain, due to pressure of the tumour on the urethra, bladder, or bowel, is common, and inimical to health. Inflammation (infection) and gangrene are dangerous conditions. Fibroids complicated with tubal and ovarian disease demand careful attention. The chief indications for surgical interference may be summarized thus : — 1. A stalked tumour protruding at the mouth of the 208 aONNECTIVE-TISSUE TUMOURS womb is readily detactied by seizing it with a volsella and twisting the stalk ; or the pedicle may be divided by scissors. 2. The presence ot a submucous fibroid is often con- jectural ; then the cervical canal is dilated and the interior of the uterus explored with the finger. Small fibroids thus discovered are easily removed. Larger tumours require enucleation. 3. Submucous fibroids with a diameter exceeding 5 to 6 centimetres usually require removal of the uterus (hyster- ectomy). There is a consensus of opinion among surgeons and gynaecologists that hysterectomy for fibroids may be recom- mended in the following circumstances : — i. When the fibroids cause j^rofuse and long-continuing menorrhagia. iL When the fibroid is septic and gangrenous. iii. Impacted and irreducible fibroids causing pain and retention of urine, iv. Fibroids which are growing rapidly and those which are degenerate and softened (cystic). v. Cervix fibroids too large to permit of removal b}^ the vagina, vi. Fibroids complicating pregnancy, delivery, and puer- pery under certain conditions. It is admitted by most writers that the ideal method of dealing with fibroids requiring removal by coeliotomy is to remove them either by ligature or by enucleation, and in certain circumstances b}?- actually opening the uterine cavity, extracting the tumour, and then suturing the incision as after a Csesarean section, an operation to which I applied the term hysterotomy. In actual practice this ideal operation of re- moving the tumours and leaving the uterus and ovaries intact can only be carried out in a small proportion of cases, probably in less than 10 per cent., and it is fair to state that enucleation and hysterotomy are often more troublesome and serious operations than hysterectomy; also the preservation of the uterus is not always an advantage to the patient. When a woman is submitted to hysterectomy for fibroids we can assure her that the tumours will not recur, but after TREATMENT OF UTERINE FIBROIDS 209 a myomectomy or an enucleation performed during the pro- ductive period of life we cannot give her this assurance, for she may have in her uterus many " seedlings/' or what I prefer to call "latent fibroids," and one or several of these may grow into formidable tumours. In the case of a young woman contemplating matri- mony, or a married woman anxious for offspring, myomec- tomy is a justifiable operation. Experience, however, teaches this stern lesson : After the enucleation of a fibroid in the procreative period of life a woman is more likely to groiu more fibroids in her ivomb than to conceive successfully. Another legitimate class of case in which myomectomy is a very safe undertaking is in patients at or after the menopause, where a stalked fibroid gives trouble by twist- ing its pedicle, or by shrinking to such a size that it falls into the true pelvis and becomes impacted ; or, more rarely, where the pedicle entangles a loop of small intestine and obstructs it. There are two methods of removing the uterus in the radical treatment of fibroids, namely, vaginal hysterectomy and abdominal hj^sterectomy. Vaginal hysterectomy is only applicable when the tumour is small or septic. There are two methods of removing the uterus by the abdominal route : one known as subtotal hysterectomy, in which the body with a variable portion of its neck is re- moved ; and total hysterectomy, in which the body and neck of the uterus are completely removed. Subtotal hysterectomy is a simpler operation than re- moval of the whole uterus. When carefully performed, within a few weeks of the operation the stump is movable, and the vaginal vault free and undamaged, and the condi- tion of the parts is such that by digital examination or inspection it would be difficult to determine that the patient had lost her uterus. The disadvantage uro-ed ag'ainst this method is the liability of the stump to be attacked by cancer. A critical examination of the reported cases shows that in some of them an unsuspected cancer existed at the time of the primary operation (Chap, xxxviii.). Total hysterectomy is a severe procedure, and attended 210 CONNECTIVE-TISSUE TUMOURS often with more shock than the subtotal operation ; it is also attended with risk of injury to the vesical segments of the ureters. Experience teaches that subtotal hj^sterectomy in spinsters or barren married women, when the uterus has a long nar- row neck and an undilated cervical canal, is as safe as any major operation in surger}^. Total hysterectomy should, as a rule, be reserved for those who have had children, and in whom the cervical canal is patulous, perhaps septic, and in many cases large and hard, or large and spong}^ If there be the least sus- picion of malignancy associated with the tumour, then com- plete removal of the cervix with the uterus is imj)erative. Careful observations have been made on women who have submitted to hysterectomy for fibroids, and they prove that the operation is followed by a remarkable improvement in the general health of the patients. Since the introduction of subtotal hysterectomy for troublesome fibroids the risks of the operation have greatly diminished. In some hospitals in London the mortality has fallen to less than 2 per cent. Bland-Sutton, J., "The Position of Abdoraiual Hysterectomy in London," 1910. From the tooth- follicle. GROUP IL TUMOUR-DISEASES OF TEETH CHAPTER XXI ODONTOMAS AND DENTAL CYSTS An odontoma is a tumour composed of dental tissues in varying proportions and different degrees of development, arising from teeth-germs, or from teeth still in the process of growth. The species of this genus are determined according to the part of the tooth-germ concerned in their formation. 1. Epithelial odontoma : from the enamel-organ. 2. FoUicuiar odontoma 3. Fibrous odontoma 4. Cementoma 5. Compound follicular odontoma 6. Radicular odontoma: from the papilla. 7. Composite odontoma : from the whole germ. 1. Epithelial odontomas. — These tumours occur, as a rule, in the mandible, but they have been observed in the maxilla. They have a fairly firm capsule, and in section display a congeries of cysts of various shapes and sizes ; but the ioculi rarely exceed 2 cm. in diameter. The cysts are separated by thin fibrous septa, sometimes ossified. The cavities contain brown mucoid fluid. The growing portions of the tumour have a reddish tint (Fig. 113). Histologically, an epithelial odontoma consists of branch- ing and anastomosing columns of epithelium, portions of which form alveoli (Fig. 114). The cells occupying the alveoli vary ; the outer layer may be columnar, whilst the central cells degenerate and give rise to tissue resembling the stel- late reticulum of an enamel-organ. 211 212 ODONTOMAS These tumours have been investigated by Eve (who gave them the name of multilocular cystic epithehal tumours) and by Falckson and Bryck. Some of the tumours, as these observers think, may arise in persistent vestiges of enamel- organs. A careful re-examination of a few of the specimens described as multilocular cystic epithelial tumours of the jaws, and a study of the descriptions of others, especially those occurring in individuals past middle life, have satisfied me that many of them were endotheUomas : some of the most typical examples of these tumours arise m the gums. Endotheliomas present such peculiar characters, structural Fig. 113. — Epithelial odontome. (jVat. size.) and clinical, that they need a group to themselves. They are dealt with in Chap. XL. (p. 405). This view of the matter is confirmed by the fact that some of these cystic tumours of the jaw supposed to arise in belated rudiments, or vestiges, of enamel-organs display malignancy, inasmuch as they recur after removal. Moreover, these tumours occur in individuals at, or after, mid-life, whereas if they arise in epithelial vestiges of the enamel- organ they ought, theoretically, to be met with in the young, which is not the case. A careful reperusal of the clinical histories of the cases collected by Heath convinces me that in the majority of instances these tumours arise in con- nexion with the mucous membrane of the jaws. 2. Follicular odontomas. — This species comprises those swellings often called dentigerous cysts, a term which has come to be used so very loosely that it should be discarded FOLLIGULAR ODONTOMAS 213 in the necessity for precision. Follicular odontomes arise commonly in connexion with teeth of the permanent set, and especially with the molars ; sometimes they attain large dimensions, and produce great deformity, especially when they arise in the upper jaws and happen to be bilateral. They occur in connexion with supernumerary teeth. The tumour consists of a wall of varying thickness, which represents an expanded tooth-follicle ; in some cases it is thin and crepitant, in others it may be 1 cm. thick. The cavity of the cyst usually contains viscid fluid and the crown or the root of an imperfectly developed tooth ; occasionally the tooth Fig. 114. — Microscopical characters of an epithelial odontome. is loose in the follicle, sometimes inverted, and often its root is truncated (Figs. 115 and 116); exceptionally the tooth is absent, or represented by an ill-shaped denticle. The walls of the cyst usually contain calcific or osseous matter; the amount varies considerably. Some observers have noted the presence of an epithelial lining to the inner walls of follicular odon- tomas : it is a point which requires further elucidation. In two recent specimens I failed to find an epithelial lining. These tumours are not unknown in other mammals; I have seen them in sheep, pigs, and porcupines. In sheep they are common, and generally affect the incisors, and are thus limited to the mandible : as a rule they are bilateral. The amount of fluid in a follicular odontoma varies, and the size of the tumour depends in the main upon this. Occa- sionally the fluid may measure as much as two ounces, and 214 ODONTOMAS this may lead to the wide separation of the inner and outer plates of the body of the mandible, and the odontoma may occupy the whole length of the bone. (Fearn's case, preserved Fig. 115. — Follicular odontomas from the mandible. in the museum of the Royal College of Surgeons, is a good example of this condition.) Hopewell Smith found that a tooth from a follicular odontome had no Nasmyth's membrane, and suggests that the fluid within these tumours is probably formed from the degeneration and liquefaction of the stellate reticulum. Fig. 116.— Follicular odon- tome from the right half of a mandible, removed from a boy aged 14 years, by Wormald, 1850. {Museum of the Royal Col- lege of Surgeons.) Fig. 117. — Fibrous odontome from a goat. {Nat. size.) Tomes has suggested that this species of odontome is probably due to the excessive formation around a retained tooth, between it and the wall of the follicle, of a fluid which FIBROUS ODONTOMAS 215 is normally present after the complete development of a tooth. Many teeth are retained without cysts forming around them, so that something beyond mere retention of a tooth is necessary for the production of a follicular odontoma 3. Fibrous odontomas. — In a developing tooth, a portion of the connective tissue in which it is embedded is found to be denser and more vascular than the rest ; it also presents a fibrillar arrangement. This condensed tissue is known as the tooth-sac, and when fully developed presents an outer firm wall and an inner looser layer of tissue. At the root of the tooth the follicle-wall blends with the dentine papilla, and is indistinguishable from it. Before the tooth cuts the gum it is completely enclosed within this capsule. Under certain conditions this capsule becomes greatly increased in thickness, and so thoroughly encysts the tooth that it is never erupted (Fig. 117). Such thickened capsules are mistaken for fibrous tumours, especially if the tooth be small and ill-developed. Under the microscope they present a laminated appearance, with strata of calcareous matter. To these the term fibrous odontomas may be applied. They are more common in rumin- ants than in other mammals, and are especially frequent in goats. As a rule they are multiple, four being by no means an unusual number. They occur in marsiipials, bears, and lions, as well as in man. There is good reason for the belief that rickets is responsible for some of these thickened capsules. Certainly, in some of the most typical examples which have been observed in human beings the subjects were rickety children ; the bilateral tumours, in some cases, being erroneously described as myeloid sarcomas. 4. Cementomas.— When the capsule of a tooth becomes enlarged, as in the specimens just considered, and these thick capsules ossify, the tooth will become embedded in a mass of cementum. To this form of odontoma the name cementoma may be applied. Tumours of this character occur most fre- quently in horses, and sometimes attain a large size. Broca has described and figured specimens from horses. Tomes has described one which weighed ten ounces, and I have given an account of another which weighed twenty-five ounces (Fig. 118). When it was divided, three teeth could be made out, 216 ODONTOMAS embedded in cementum. The periphery of the tumour was cautiously decalcified in hydrochloric acid, and sections were prepared for the microscope. The structure of the de- calcified mass was very instructive, for the periphery of the tumour exhibited the laminated disposition seen in fibrous odontomes. The largest cementoma from a horse known to me is preserved in the museum of the Koyal Veterinary College, London; it weighs seventy ounces, and, though excessively dense, is nevertheless very vascular. Its chief structural Fig. 118. — Cementome from a horse. {JIalfnat. size.) {Museum of the Royal College of Surgeons.) peculiarity is the presence, in enormous numbers, of large, richly branched lacunse. 5. Compound follicular odontomas. — If the thickened capsule ossifies sporadically instead of uniformly a curious condition is brought about, for the tumour will then contain a number of small fragments of cementum^ or dentine, or even ill-shaped teeth (denticles) composed of three dental elements — cementum, dentine, and enamel. The number of teeth or denticles in such tumours varies greatly, and may reach a total of four hundred. Tumours of this character have been described in the human subject by several observers. Amongst the most noteworthy are the following. COMPOUND FOLLICULAR ODONTOMAS 217 Tellander, of Stockholm, met with a case in a woman aged 27 years. The right upper first molar, bicuspids, and canine of the permanent set had not erupted, but the spot where these teeth should have been was occupied by a hard, painless enlargement, which the patient had noticed since the age of 12 years. Subsequently this swell- ing was found to contain minute teeth. There were nine single teeth, each one perfect in itself, having a conical root, with a conical crown tipped with enamel ; also six masses built up of adherent single teeth. The denticles presented the usual characters of supernumerary teeth (Fig. Fig. 119. — Odontome (cementoine) from the mandible of a rickety youtli aged 19 years. A, denticle ; b, portion of the outer wall of the jaw. 120, a). About a year afterwards a tooth appeared in the spot from which this host of teeth was removed. A similar case has been recorded by Sir John Tomes, the details of which were communicated to him by Mr. Mathias, on medical service in India. A Hindoo aged 20 had a large tumour in his mouth containing a number of ill-formed teeth; fifteen masses of supernumerary teeth and bone were removed from it. The soft parts rapidly healed, the deformity disappeared, and subsequently the only peculiarity noticeable was the absence of the central and lateral incisors. The canines occupied their usual position. A third example of this remarkable condition has been recorded by Windle and Humphreys. The tumour was found in the mouth of a boy aged 10 years ; neither the deciduous nor the permanent right lateral incisor or canine had erupted, The space thus unoccupied was filled by a 218 ODONTOMAS tumour with dense, unyielding walls. From this tumour forty small denticles of curious and irregular forms were removed. Ward Cousens has described one of these remark- able tumours which grew in a boy aged 11 years. He re- moved at various times one hundred denticles. They are preserved in the museum of the Royal College of Surgeons. Fig. 120. — A, Denticles from Tellander's case. Total number, twenty-eight. E, ,, from Windle's case. Total numter, forty, c, „ from Mathias's case. Total number, fifteen. Hildebrand and De Roaldes have observed similar cases. Logan reported an example from the maxilla of a horse con- taining four hundred denticles ; and in a Himalayan goat the writer found one of these singular tumours in each upper jaw, containing nearly three hundred denticles. This specimen is preserved in the museum of the Royal College of Surgeons. 6. Radicular odontomas. — This term is applied to odon- tomes which arise after the crown of the tooth has been completed, and while the roots are in the process of form- RADIO ULAB ODONTOMAS 219 ation. As the crown of the tooth, when once formed, is unalterable, it naturally follows that, should the root develop an odontome, enamel cannot enter into its composition ; the tumour would consist of dentine and cementum in varying proportions, these two tissues being the result of the activity of the papilla. As a typical radicular odontome we may choose the well- Fig. 12) .—Radicular odontome from human subject, a represents the natural size of the specimen. (After Salter.) known specimen described by Salter (Fig. 121), in which the tumour is clearly connected with the roots. The outer layer of the odontoma is composed of cementum ; within this is a layer of dentine, deficient in the lower part of the tumour, and inside this is a nucleus of calcified pulp. A radicular odontome (Fig. 122) described by Sir John Tomes in 1863, and redescribed by Mr. C. S. Tomes m 1872, consisted of a mass invested by cementum ; inside this casing IS a shell of dentine ; the tubules radiate outwards and are disposed with some regularity : this dentine was deficient at 220 ODONTOMAS the distal end of the tumour ; its interior was filled with an ill-defined osseous material. I removed an odontome from a boy 15 years of age. An accurate diagnosis was made before the operation with Fig. 122. — Radicular odontome, removed from the upper jaw of a man aged 41 by Mr. Hare. (iV«^. size.) {After Sir John Tomes.) the assistance of the X-rays (Figs. 123 and 124). The tumour consisted of bone resembling that which forms the alveolar borders of the jaws, embedded in fibrous tissue. As shown in the drawings, the second left mandibular molar Fig. 123. — A, Odontome suiToundiug the second left mandibular m.olar of a boy aged 15 years. B B, The odontome in section, show- ing the relation of the roots to the tumour-tissue. below the neck of the tooth seems to expand and become gradually incorporated with the tissue proper of the odontome. It is probable that some radicular odontomes in man are due to inflammatory changes ; for example, the tumour- like swelling enveloping the roots of two molars (Fig. 125) RADICULAR ODONTOMAS 221 supports this view. The roots are embedded in an ossific ball which microscopically resembles a calcified and partially ossified inflammatory exudate. The crown of one of the teeth carious, and the pulp-chamber widely exposed. This IS Fig. 124.— Skiagram showing the odontome illustrated in Fig. 123. tumour was removed by Mr. Murray from the mandible of a youth aged 21 years. The curious odontome shown in Fig. 126 was extracted from a Chinese student in Hankow by Mr. Davenport. There was a swelHng around the tooth supposed to be due to an abscess. On a casual examination the lump on the root of 222 ODONTOMAS this second lower molar appears as a radicular odontome, but on section it presents the complex structure of a composite odontome. The clinical report contains the significant state- ment that there were no signs of the lower wisdom teeth. Fig. 125. — Ossific ball in which the roots of two molars are embedded. The crown of one tooth is carious, a, The tumour entire ; B, in section. [JIuseum of the Middlesex Sospital.) Radicular odontomes have been observed in the marmot, the porcupine, the agouti, and the boar (Figs. 127, 128), and in elephants. It is very probable that many, perhaps most, of these thickened roots of tusks in boars and elephants and the rig. 126. — Second right mandibular molar of a Chinaman aged 19 years, with a tumour possessing the characters of a composite odontome. a and b, The tooth of natural size ; c, the tooth enlarged and the tumour shown in section. incisors of rodents are due to inflammatory changes in the pulps. 7. Composite odontomas. — This is a convenient term to apply to those hard tooth -tumours which bear little or no resemblance in shape to teeth, but occur in the jaws, and COMPOSITE ODONTOMAS 223 consist of a disordered conglomeration of enamel, dentine, and cementum. Such odontomes may be considered as arising from an abnormal growth of all the elements of a tooth- germ — enamel-organ, papilla, and follicle. Fig. 127. — Left lower jaw of a young marmot with a large radicular odontome connected with the incisor. (Nat. size. ) Not only is this species of odontoma composite in that the tumours comprised in it originate from all the elements of a tooth-germ, but they are composite in another sense : many of these tumours consist of two or more tooth-germs in- discriminately fused. But they differ from the cementomas containing two or more teeth in the fact that the various parts of the teeth composing the mass are indistinguish- Fig. 128. — Radicular odontome connected with the mandibular canine of a boar. ably mixed, whereas the individual teeth implicated in a cementoma can be clearly defined. Forget's classical case belongs to this species. The patient was 20 years old, but the disease had been noticed since 224 ODONTOMAS tlie age of 5 j^ears. Behind the first bicuspid no teeth were seen, but the jaw as far back as the ramus was the seat of a smooth, unyielding tumour (Fig. 129), which consisted mainly of dentine ; its surface was in places covered with enamel. It was long believed that composite odontomas occurred only in the mandible ; now that we know more about them, it is clear not only that they arise as frequently in the maxillse, but that they attain a far larger size in the upper than in the lower jaw. In the mandible these tumours Fig. 129. — Composite odontome. {yat. size.) {After Foryef.) may attain to a large size. One of the largest (Fig. 130) was removed by Mr. Brothers, of Cape Town, from a Kaffir boy aged 14 years. The parents of the boy stated that they "noticed a swelling when the boy was 6 months old." He ran about the village with part of the tumour sticking out of his mouth : it was extracted with a strong elevator. Many large odontomes removed from the antrum have been described as exostoses. Thus, M. Michon removed from the antrum of a Frenchman aged 19 years, at the Hopital de la Pitie (without an ansesthetic), an odontome weighing 1,080 grains. The operation, which may be de- scribed as a " surgical struggle," lasted upwards of an hour and a quarter. The tumour is described as an exostosis, but fortunately Michon's account is accompanied by some COMPOSITE ODONTOMAS 225 excellent figures which show clearly enough that the tumour is of dental origin. The cut surface exhibited a laminated disposition. Microscopically it was composed of tissue pre- senting many parallel tubules having the appearance of exaggerated dentinal tubules. A tumour almost parallel with this has been de- scribed by Dr. T. Duka, by whom it was removed from a Mahomedan woman aged 26, at Monghyr, Bengal. The woman had for six years suffered from a muco-purulent Fig. 130. — Composite odontome from the mandible of a Kaf&r boy 14 years of age. (850 grains.) {^Ihsciou, Roi/al Dental Hospital, London.) discharge from the right nostril, and was anxious for relief. The case was regarded as one of necrosis, but after a"sur- gical struggle" lasting nearly an hour (without chloroform), the tumour (Fig. 131) was withdrawn from the antrum. It had no connexion with the surrounding tissues. The "tumour, which was regarded as an exostosis, was sub- mitted to a committee of the Pathological Society. In its report the committee states that the osseous tissue differs in character from that ordinarily seen in exostoses. An examin- ation of the tumour, which is preserved in St. George's Hospital museum, and inspection of the figures illustrating the report mentioned, show clearly enough that it is a com- posite odontoma. Dr. Duka, in his account of the case, states that Dr. Allen Webb was of opinion that the nucleus was formed by a tooth-follicle escaping into the antrum of High- more. This was a guess, but one not far short of the truth. 226 ODONTOMAS The largest odontooie known to have grown m the human antrum, and which Hilton described as an exostosis, is pre- served in the museum of Guy's Hospital (Fig. 133). It has an extraordinary clinical history: — A man, aged 36 years had a large osseous tumour occupying the antrum. The pressure of this tumour had caused the front wall of the antrum, with the integu- ment and soft tissues covering it, to slough. The trouble was first noticed thirteen years before: as the cheek en- larged the eyeball became displaced and finally burst. For a long time the surface of the tumour was exposed, the suppuration being co]3ious, and occasionally pieces of bone Fig. 131. — Composite odontome from the iipi^er jaw. (Ji^at. size.) irregular in shape came away ; at last, to the man's astonish- ment, the bony mass drojaped out, leaving an enormous hole in his face. It weighed nearly 15 ounces, and measured 27 "5 cm. (11 inches) in its greatest circumference. I have had an opportunity of investigating this tumour ; it is remarkably hard, presents on section an ivory-like surface, and, on close scrutiny, a number of closel}^- arranged concentric laminae (Fig. 134). Sections ground thin and examined under the microscope show large numbers of lacume and canaliculi arranged in a veiy regular manner. On looking over a long series of composite odontomas it is curious to find the great variety in shape, as well as in the Fig. 132. — Group of odoutomes. A. Upper jaw (Brock). B. Lower jaw (Rushton Parker). C. Ujjper jaw (Jordan Lloyd). D. Lower jaw (Windle). E. Radicular odontoma (J. G. Turner). 227 228 ODONTOMAS disposition of the liard dental tissues, which they present. The specimen represented in Fig. 135 is one of the oddest in this respect, for it in no way recalls in its shape a tooth, yet the whole of its convex surface (for it is shell-like in form) is occupied with well-marked enamel-covered projections resem- bling small supernumerarj^ cusps on teeth. This tumour came from an old woman, an inmate of a workhouse ; she had been Fie 133. — Large odontome -vrliich was spontaneously shed from the antrum ; weight nearly 15 ounces. {Museum, Guy''s Hospital..) troubled with it for very many years, and one day she " spat it out." Clinical characters. — The germ of any permanent tooth may develop into an odontoma, and occasionally two or more teeth may be involved in the one tumour. Odontomes occur with equal frequency in the upper and lower jaws. The follicular species is very apt to be multiple, and four have been found concurrently in the jaws of the same patient. The composite species ranks next in frequency. In the upper jaw an odontome may invade the antrum and attain the size of a child's fist ; in the mandible it rarely exceeds a dove's CLINICAL CHABAGTERS 229 Bgg in size, though in this situation an oclontome may at- tain a good size (Fig. 130). There is a chnical point in the natural history of odon- tomes of some importance. A careful examination of the clinical history shows that in nearly all cases the tumours have remained quiescent, and then there comes a period in which, like teeth, they seeni to erupt and make their way above the gum, and very often cause profound constitutional disturbance, mainly of a septic character. In some reported cases it is stated that the patients have been so ill as to be Fig. 134. — Section of tumour shown in Fig. 133, exhibiting concentric lamination. near death. This phenomenon usually happens between the twentieth and the twenty-fifth years. In a fair number of cases relief has come to the patient and the illness has ended by the tumour loosening spontaneously. In several instances it is said that the patient "spat it out." This happened with the specimens, Figs. 132, d, and 135. One of the largest odontomes known, after producing hideous deformity of the face and sloughing of the cheek, fell out of its own accord. The diagnosis of these tumours has been a matter of Q-reat difficulty in the past. In many the swelhng has been regarded as a myeloma, or a sarcoma, but in the majority of cases, especially where there has been free suppuration around the tumour, it has been regarded as a piece of dead bone. The X-rays have been serviceable in enabling a correct diagnosis to b.e made {see Fig. 124). 230 ODONTOMAS Treatment, — A study of the literature relating to the treatment of odontomas is very instructive, because it re- veals that operations unnecessarily severe have been under- taken, in ignorance of the nature of the disease, by surgeons of high reputation and Avide experience. In several instances it is known that a great portion of the mandible has been excised under the impression that the tumour was malig- nant in nature. In some verv large odontomes of the upper jaw the tumour has been removed without an anaes- thetic, the procedure being described, in the words of the operator, as " a surgical struggle " (Duka, Michon). In some A B Fig. 135. — Composite odontomes from the mandible: A, the upper, B, the lower view. {Museum of the Middlesex Hospital.) of the cases dentists succeeded in removing the tumour with forceps, thinking they were dealing with unerupted (buried) teeth (Davenport), In other instances the nature of the tumour has been suspected, but in the course of its excision the mandible has been broken and has remained permanently ununited. In the case of a tumour of the jaw the nature of which is doubtful, particularly in a young adult, it is incumbent on the surgeon to satisfy himself, before proceeding to excise a portion of the mandible or maxilla, that the growth is not an odontome, for this kind of tumour only requires enucleation. In the case of a follicular odontome it is very essential to remove the sac completely. Dental cysts. — It occasionally happens in extracting per- manent teeth that a small fibrous bag is found at the apex of DENTAL GYSTS 231 the root, usually no larger than an apple pip, though some- times it may be as big as a bantam's egg, filled with fluid, and often containing crystals of cholesterin. These sacs, or dental cysts, occur in connexion with the dead roots of mandibular and maxillary teeth, especially molars and pre- molars. They sometimes attain a considerable size in the upper jaw when they invade the antrum, and some of these cysts are sufficiently large to simulate an abscess of the cavity. Dental cysts are often bilateral, and occasionally multiple. The constant association of these cysts with the dead roots of permanent teeth has led many observers to regard them as pus- sacs with thick, fibrous walls. Mr. J. G. Turner has carefully investigated their struc- ture, and demonstrated the existence of an epithelial lining in many dental cysts. He believes that they arise in the " rests " detected by Malassez and known as para- dental epithelial remnants. They are de- rived from a prolongation of the enamel- organ which precedes and determines the formation and shape of the dentine and the root of the tooth. I have had several dental cysts ex- amined microscopically, and can confirm Turner's observa- tion that they possess an epithelial lining. The epithelium is usually stratified, but columnar cells occur, especially in cysts associated Avith the first permanent molar. The restriction of these cysts to the roots of the permanent teeth is explained by the fact that the roots of temporary teeth as Avell as their alveoli are absorbed. The majority of dental cysts are met with accidentally in extracting dead permanent teeth or their roots. Large specimens, however, resemble in their clinical signs tumours of the jaws or antrum (Fig. 137). Even cysts of the size of a dove's egg in relation with the lower molars and pre- molars will so expand the outer plate of the mandible as to yield parchment-crackling on being firmly pressed with the finger. When a painless smooth tumour of the jaw is Fig. 136.— Dental cysts at the roots of a dead lower molar. 232 ODONTOMAS associated with a carious tooth, especially of long standing, a dental cyst should be borne in mind. The association ot" these cysts with carious and dead teeth is sufficient to pre- vent them from beinsf mistaken for follicular odontomes. Fig. 137.— Large cyst connected with the mandible ; it is probably an unusually large dental cyst. [Muscmn, St. George''s Hosjntal.) Treatment. — The roots must be extracted, and the cyst- wall thoroughly enucleated, and the cavity stuffed with ste- rilized gauze and allowed to granulate. If any part of the cyst-wall be allowed to remain it will lead to a persistent and usually troublesome sinus. REFERENCES 233 Bland-Sutton, J., "A Yeiy Large Oclontome from a Horse." — Trans. Odont. Sac. Gt. ISrlt., 1891, xxiii. 215. Bland-Sutton, J., "An Anomalous Tumour from the Antrum." — Ihid., 190'i, xxxiv. 9G. Bland-Sutton, J., " On a Radicular Odontoma from the Mandible." — Ibid., 1906, xxxviii. 19. Broca, Paul, " Odontomes." — " Traite des Tumeurs," 1869, ii. 350. Colyer, J. F., " On some Odontomes and Anomalous-shaped Teeth in the Museum of the Society ."—Trans. Odont. Soc. Gt. Brit., 1906, xxxviii. 245. Cousens, W. J., "A Case of Compound Follicular Odontoma." — Lancet, 1908, i. 1352. Duka, T., "A Bony Tumour of the Nasal Fossa." — Tra-ns. Path. Soc, 1866, xvii. 256. Eve, F. S., "On Cystic Tumours of the Jaws.''— Brit. Med. Journ., 1883, iii. 1. Heath, C, " Injuries and Diseases of the Jaw." Hildebrand, Zeitschr.f. Chir., 1891, xxxi. 282. Hilton, J., "A Large Bony Tumour of the Face." — Guy's Hasp. Eepts., 1836, i. 493. Hopewell-Smith, A., "Two Odontoceles and some other Cysts." — Proc. lioy. Soc. of Med., Odont. Sec, 1910, iii. 121. Mathias, "A Group of Supernunaerary Teeth.." --Trans. Odont. Soc. Gt. Brit., 1863, iii. 365. Michon, " Exostose eburnee du Sinus maxillaire." — Mem. de la Soc. Chir., Paris, 1850, i. 608. de Roaldes, " A Compound Follicular Odontome." — New Yorh Med. Journ., 1894, lix. 612. Smale, Morton, Trans. Odont. Soc. Gt. Brit., xxxv. Tomes, Chas. S., "Description of an Odontome." — IMd., 1872, iv. 81. Tomes, Chas. B.~Ibid., 1872, iv. 103. Tomes, Sir J., " A Remarkable Case of Exostosis." — Ibid., 1863, iv. 335. Windle, B., and Humphreys, J., " A Rare Tumour connected with the Teeth." — Journ. of Anat. and Phys., 1887, xxi. 667. GROUP III. EPITHELIAL TUMOURS CHAPTER XXII PAPILLOMAS (WARTS) Ix the group of tumours now to be considered, epithelium is not only present, but is the essential and distinguishing- feature. Epithelium is so disposed in the bodies of complex animals as to serve many functions : in some situations it acts as a protective — e.g. the epidermis, where it becomes modified into hair, nail, horn, or into the hardest of all animal tissues — enamel; in others, epithelial cells dip into the underlying connecting tissue to form secreting glands; some of them are simple — e.g. the tubular glands of the intestine ; others are very complex — e.g. the liver, mamma, and kidney. Whether a gland is simple or complex, the principle of its construction is identical— namely, narrow channels lined with epithelium, resting upon a connective- tissue base, in which blood-vessels, lymphatics, and nerves ramify. Each e]3ithelial recess of a gland is known as the acinus, and each acinus is in communication with a free surface, either directly by its own duct, as in the case of seba- ceous and mucous glands, or indirectly by means of a number of main ducts, as in the case of the mamma; or by a common duct, as in the pancreas. To this rule there are exceptions : the thyroid gland, the pituitary body, and the ovary. The various members of the epithelial group of tumours fall readily into three genera: 1, papillomas (warts); 2, ade- nomas ; 3, carcinomas (cancers). In this and the next chapter papillomas and horns will be considered, the other two genera being dealt with in later chapters. 2U PAPILLOMAS 235 PAPILLOMAS A papilloma or wart consists of an axis of fibrous tissue, containing blood-vessels, surmounted by epithelium, projecting from an epithelial surface ; it may be simple, and present a uniform surface, or be so covered with secondary processes as to look like a mulberry. When the processes are long the papilloma has a villous appearance. Fig. 138. — Horn which grew from a wart on the cheek of a very old Welsh woman ; it measured 21 cm. along its greater curve. Warts are most common on the skin, but they also arise from mucous surfaces covered with squamous epithe- lium. They occur singly or in multiples ; occasionally they are thickly crowded on a restricted area of the skin, and form a wart-field. Warts are rarely painful unless irritated, then they are apt to ulcerate and bleed. Crops of warts are often seen on the hands of children. They are common in the region of the anus, vagina, and glans penis when these parts are irritated by foul discharges, especially those of gonorrhceal origin. A curious feature of multiple warts is that they sometimes appear on the hands or scalp almost 236 EPITHELIAL TUMOURS suddenly, and after persisting many weeks, or perhaps months, disappear as if by magic. When warts are thickly crowded upon a limited area of skin — as, for example, the glans penis— they may be mistaken for a more serious dis- ease, such as wart}^ carcinoma. When they appear in great number, they are due to local infection by micro-organisms. Skin-warts are overgrown papilla3, and on section the epithelium will be found to pass from one papilla to another in an unbroken line without invading the fibrous frame- work. A solitary wart may occur on any skin-covered surface and persist. A wart of this character sometimes attains the size of a walnut, and in some cases is mottled with black pigment. Such warts, late in life, may become the starting- points of melanomas. Occasionally one or two sparse hairs may be detected on a wart ; and some fun is made out of this fact by Cressida when her uncle Pandarus says con- cerning the glabrous chin of Troilus: " And she takes upon her to spy a white hair on his chin." Cressida replies : " Alas, poor chin ! Many a wart is richer." Troilus and Cressida, i. 2. Solitary warts sometimes grow rapidly, and become so large that they are apt to be mistaken for malignant tumours. They are red, like the comb of a cock, and smeared with purulent material, and often very fetid. Billroth drew attention to large, rapidly growing tumours of this kind arising in soft warts of the face, and termed them plexiform sarcomata (see also McCarthy and Bland- Sutton). Warts growing from the skin are covered with squamous epithelium, and the surface cells are liable to be transformed into horny material and form what are called cutaneous horns. Some of these wart-horns have attained almost fabulous dimensions (Fig. 138). Warts are by no means uncommon in domesticated animals. They are frequent on the penis of horses and bulls, the lips of lambs, and the PAPILLOMAS 237 pads on the feet of dogs and cats and cat-like mammals (Felida)). Warts similar in structure to those of the skin occur on mucous membranes with a covering of squamous epithelium, such as the lips, buccal aspect of the cheeks, vestibule of the nose, and the larynx. The oesophagus of the ox is occa- sionally the seat of a multitude of dendritic warts. Laryngeal warts. — In the larynx, warts most commonly spring from the mucous membrane covering the true cords ; Fig. 139. -Larynx of a child opened jjosteriorly ; it is full of warts, from suffocation. The child died frequently they grow immediately beneath the cords, and a not uncommon situation is immediately below the point of attachment of the vocal cords to the thyroid cartilage. Ex- ceptionally a large mulberry-like wart has been detected growing from the floor of the sinus pyriformis. In number laryngeal warts vary greatly. Often but one is present; in other cases ten or more will be found. In size there is great differ- ence : some Avarts are not larger than the head of a pin ; they rarely exceed the dimensions of a small cherry, and as a rule they are no bigger than split peas. The warts may be sessile or pedunculated; in the latter case they sometimes possess great mobility, and get nipped between the vocal cords and 238 EPITHELIAL TUMOURS o-ive rise to urgent dyspnoea, which occasionally ends in suffocation (Fig. 139). In colour they are of a delicate pink, sometimes of a whitish tint resembling that of the healthy cords. Haemorrhage into their substance causes them to assume a deep -red tint. Laryngeal warts occur in children and adults. A curious feature connected with them in children is their disappear- ance after tracheotomy. This is similar to the sudden manner in which warts on the skinsome times vanish. Intracystic warts. — This variety of papilloma frequently grows in mauniiary cysts, especially those which arise in the sinuses of the galactophorous ducts. Warts of this kind are associated with the disease known as duct-cancer of the breast (see Chap. xxix.). The remarkably luxuriant papillomas which arise in the cysts of the hilura of the ovary are described in the section dealinsr with Tumours of the Genital Glands. Papillomas grow in the small cysts formed by dilatation of sweat-glands. They have been observed in the axilla (Robinson) and on the cheek (Rolleston). Adams has made a careful histologic examination of the cysts which arise in the condition known as hydro- cystoma. These cysts, which do not exceed in size barley- corns, are limited to the face, and arise from an abnormal dilatation in the coil of the sweat-glands. The epithelial lining of such cysts is always very rich, and they sometimes contain intracystic growths. Villous papillomas. — These grow from the mucous membrane of the bladder, the renal pelvis, and the choroid plexuses of the ventricles of the brain. In the bladder the general appearance of the long, branching, feathery tufts recalls in a striking manner the delicate chorionic viUi, and when viewed with the cystoscope in the living bladder they are often exquisite objects. They consist of a connective-tissue core traversed by delicate blood-vessels, the whole being surmounted by epithelium. These villous growths sometimes have broad bases, but in other cases the points of attachment are so narrow that the tumours may be described as pedunculated. Usually villous tumours of the bladder occur singly, but two, three, or more PAPILLOMAS 239 may be fonnd in the same bladder. Occasionally there is one large villous tuft with several smaller masses of the size of peas. In some instances they occur at or near the orifice of the ureter, and, though small, the tumour will give rise to serious changes in the corresponding kidney by obstructing the flow of urine from the ureter. When the papilloma is situated near the neck of the bladder the long villous tufts will sometimes be carried by an overflowing current of urine Fig. 140. — Villous papilloma of the bladder. into the urethral orifice, and cause imjiediment to its free escape (Fig. 140). The delicate character of the villi and their vascularity are sources of danger, because the processes themselves are sometimes torn, and the hsemorrhasre is occa- sionally so severe as to place life in great peril. Villous growths in every way identical with those found in the bladder are sometimes found growing from the pelvis of the kidney (Fig. 141). In one very striking case of this sort recorded by Murchison the pelves of the kidneys of a man 65 years of age were found thus occupied, and a singular feature of the case was the presence of two villous 240 EFITHELIAL TUMOURS tumours in tlie bladder, one at the orifice of each ureter. It is not improbable, from what we know of the habits of warts generally, that in this exceptional instance the vesical warts were due to transplantations of epithelium from the pelvis of the kidney to the mucous membrane of the bladder. The passage of detached villous tufts down the ureter caused Fig. lil. — Kidney with a villous papilloma growing in its pelvis. colic like a renal calculus in a man in his eightieth year (Lendon). Villous papilloma of the renal pelvis is a somewhat rare affection, and the subjects are generally past middle life ; the condition is often bilateral, and simulates cancer of the kidnej'-. The reported cases have been collected by Nash and Savory. If care be taken to exclude cases of carcinoma of the kidney with villous tufts, true villous disease of the kidney will be found a rare condition. FAFILL0MA8 241 There is an interesting variety of villous papilloma which arises from the choroid plexuses of the cerebral ventricles. These plexuses are fringed with tufts of epithelial-covered villi which occasionally grow luxuriantly and attain a size sufficient to give rise to unpleasant effects, particularly when the choroid plexus of the fourth ventricle behaves in this manner. Douty described a case of " villous tumour of the fourth ventricle" in which the tumour was as large as a bantam's e^^ ; it obstructed the interventricular communications and led to distensions of the lateral and third ventricles ; the aqueduct of Sylvius was dilated to the size of a quill. The patient was a boy 17 years old, and the clinical features were such as to permit of accurate localization of the lesion during life. I had an opportunity of examining this speci- men. Similar cases have been reported by Clifford Allbutt, Ashby, and Briichanow. Allbutt, Sir T. Clifford, " Two Cases of Tumour of the Pons Varolii." — Trans. Path. Soc, 1868, xix. 20. Ashby, H., " Angio-Sarcoma of Left Choroid Plexus." — Trans. Path. Soc, 1886, xxxvii. 56. Billroth, Th., "Lectures on Surgical Pathology and Therapeutics." — Translated from the 8th edit. (i\^ew Syd. Soc), 1878, ii. 414-415. Bland-Sutton, J., "An Unusual Form of Wart (Plexiform Sarcoma — Billroth)." — Trans. Path. Soc, 1892, xliii. 161. Briichanow, N., " Ueber einen Fall von Papillom des Plexus Choroideus ventriculi lateralis sin. bei einem 2^ j. Knaben.'" — Prag. med. Woch., 1898, xxiii. 585. Douty, J. H., " Notes and Remarks upon a Case of Villous Tumours in the Fourth Ventricle." — Brain, 1886, viii. 409. McCarthy, Jeremiah, "Tumour of Face, Plexiform Sarcoma." — Trans. Path. Soc, 1880, xxxi. 256. Murchison, C, "Case of Villous Disease of the Bladder and Kidneys." — Trans. Path. Soc, 1870, xxi. 241. CHAPTER XXIII HORNS Cutaneous horns in the human subject are of four varieties : 1, Sebaceous horn; 2, Wart-horn; 3, Cicatrix-horn ; 4, Nail- horn. Sebaceous and wart-horns are structurally identical. It is impossible to decide from an examination of a large horn whether it grew from a sebaceous cyst or from a wart. Cutaneous horns sometimes attain great proportions, espe- cially in the aged (Fig. 138). Sebaceous horns are more frequent on the scalp than elsewhere, whilst wart-horns are most frequently found on the penis, and are not rare on the pinna. It is important to bear in mind that carcinoma is apt to originate in the skin around the bases of wart-horns, especially in elderly patients. Cutaneous horns are extremely tough, and present a longi- tudinal fibrillation; when soaked in a weak solution of liquor potassse they quickly soften, and the horny material comes away in flakes. The only means of deciding between a wart-horn and a sebaceous horn is by dividing them longitudinally, and ascer- taining the existence or otherwise of a cyst at the base of the horns. In the case of the mouse sketched in Fig. 142, some pathologists who examined it were of opinion that it was a wart-horn, but on dissection a large sebaceous cyst was found to occupy its base. Horns of this character are not rare in mice, and have been seen on a mouse which lived in a church, and on one which was caught in West- minster Abbey (W. G. Spencer). The most elaborate collection of cases illustrating cuta- neous horns is contained in a small work published by Dr. Hermann Lebert. He gives accounts of one hundred and nine cases, with references, the earliest dating from the year 242 HOBNS 243 1300. The horns were found on the scalp, temple, forehead, eyelid, nose, lip, cheek, shoulder, arm, elbow, thigh, leg, knee, toe, axilla, thorax, buttock, loin, penis, and scrotum. In length they varied from 1 to 20 cm. Lebert, however, makes no attempt to discriminate between the varieties of horns. The most curious situation in which cutaneous horns occur is in ovarian dermoids. The conversion of epithe- lium into horn in cases of sebaceous cysts and warts is Fig. 142. — Sebaceous horn in a mouse. something more than desiccation from exposure ; it is doubt- less akin to the change by which nail and horn are formed under normal conditions. A good physiological type of a cutaneous horn is pre- sented by the nasal horn of the rhinoceros. This formidable cutaneous appendage is composed of agglutinated hairs. Professor Flower exhibited at the Zoological Society, London, a portion of the skin from the head of a rhinoceros (shot by Sir John Willoughby in Central Africa) furnished with three horns. The accessary- horn was 12 cm. high and 42 cm. in circumference (Fig. 143). A physiological type of sebaceous horns is furnished by the curious patch of spines on the forearm of Hapalemur (Hcqxdemur griseus). It is present only in the adult male. The spines are identical in structure with sebaceous horns, 2U EPITHELIAL TUMOUES and are formed of hardened secretion furnislied by a mul- titude of glands in the skin immediately underlying the patch of spines. The male ring-tailed lemur (Lemur catta) has a curious hom-like spur upon its forearm near the wrist ; beneath this horny patch I found a collection of glands. Cutaneous horns are sometimes found on cows, sheep, and goats. They may attain a large size. The museum of the Royal College of Surgeons contains a very large horn that grew from the flank of a ram. It is nearly a metre in length, and in its dried condition is 28 cm. in circum- rig. 143.^Head of an African rhinoceros with a large wart posterior to and in a line with its nasal hoi^ns. ference at the base. This specimen is described, with others, by Sir Everard Home in an interesting paper (Phil. Trans., 1791). Rabelais tells us that the mare on which Gargantua rode to Paris had a little horn on her buttock. Birds are liable to cutaneous horns: they grow very rapidly, and sometimes attain great lengths. They follow the rule with regard to the epidermic structures in this class generally, and are cast oft" when the birds moult (Fig. 144). A good physiological type of wart-horn among birds is furnished by the American white pelican, P. tra.chyrhynchus. The beak of this bird is furnished with a horn structurally resembling the wart-horns occasionally seen on other birds. The horn is shed in the autumn Avhen the pelican moults. EOBNi^ 245 and is rapidly reproduced with the feathers. Mr. Spencer F. Baird states that Mr. Ridgway visited the breeding-ground of these birds on an island in Pyramid Lake, Nevada, and found the pelicans nesting by thousands. Towards the end of the season the oround became so strewn with these shed horns that they could be gathered by the bushel. Fig. 144, -Head and leg of a thrush with cutaneous horns, each time the bird moulted. The horns were cast Cicatrix - horns. — These are rare, and grow generally from the scar left by a burn. Such scars, when extensive, are liable to ulcerate and then slowly heal again, but as they heal they become covered with a mass of scales, which some- times form a horny outgrowth composed of hard desiccated tissue, often laminated like a pie-crust. Cruveilhier described a very remarkable example of this kind of horn growing from a hand, probably deformed in consequence of a burn ; the horny processes vary from 2 to 246 EPITHELIAL TUMOURS 20 cm. in length. Edmimfls has described and figured a similar specimen. Cruveilhier states that horns of this kind came under his notice on the thighs of an old woman at the Salpetriere ; the}^ grew from the scars of old burns caused by chaufferettes. When the horns became detached they left painful ulcers. Later observations show that as these ulcers heal, new horns form. Nail-horns do not call for much consideration. They are Fig. 145. — American white pelican, P. trachyrhynchus. {From a specimen in the Zoological Gardens, London.) extremely common on the toes of bedridden patients, espe- cially old women and those who are dirty. Although nail- horns may grow on any of the toes, they are most frequently met with on the big toe. The horns may attain a length of 7 cm., and become twisted so as to resemble rams' horns. Treatment. — Cutaneous horns are easily detached by a sudden jerk with the thumb and forefinger; if they are too firmly fixed to be removed in this way, then they may be excised. An exceptional case will demand amputation, and in a few instances surgeons have thought it necessary to remove the extremity of the penis when the skin surrounding the base of the horn has been ulcerated. When cancer HORNS 247 attacks the skin at the base of a horn, it should, with the surrounding skin, be early and freely excised, and the lymph- glands connected with it should be carefully dissected out, Baird, Spencer F., Ibis, 18G9, p. 350. Bland-Sutton, J., "Arm Glands of Lemurs." — Proc. ZooL Soc, 1887, p. 3G9. Cruveilhier, " Anatomie Pathologique du Corps Humain ; ou, descriptions avec figures de ses Diverges Alterations." — Morhides, 1835, pi. vi,, livraison vii. Edmunds, Walter, " Horny Papilloma of Hand." — Trans. .Path. Soc, 1887, xxxviii. 352. Flower, Sir WilJiam, Proc. ZooJ. Soc, 1889, p. 448. Lebert, Hermann, " Ueber Keratose," Breslau, 1864. Spencer, W. G., "Epithelioid Horn on a Mouse." — Trans. Path. Soc, xli. 402. Willougliby, Sir J. C, " East Africa and its Big Game," 1889, p. 155. CHAPTER XXIV ADENOMA An adenoma is a tumour constructed upon the type of, and growing in connexion with, a secreting gland. Adenomas occur as encapsuled tumours in such organs as the mamma and liver, and in glands like the parotid and thyroid ; in the mucous membrane of the rectum, intestine, and uterus they are pedunculated. A single adenoma may be present, but two or more may exist in the same gland. In the case of the intestine a score or more may grow in the same individual. In size they vary greatly : some are no larger than peas, whereas in the mamma an adenoma will occasionally attain the dimensions of a man's head. The effects of adenomas depend mainly upon the situations in which they grow. The following statements are true for all : When completely removed there is no recurrence ; they do not infect neighbouring lymph- glands, nor give rise to secondary deposits. When an adenoma causes death, it is in consequence of mechanical complications, depending on the situation and size of the tumour. Although the distinguishing structural peculiarity of an adenoma is the presence of epithelium disposed as in a secret- ing gland, the connective tissue (stroma) entering into its composition must also be taken into account. In many adenomas the epithehal element is the most conspicuous ; in others the connective tissue is out of all proportion to the epithelium, and occasionally preponderates to such a degree that the tumour from some writers receives the misleading name of " adeno-sarcoma." When the epithelium-lined spaces are distended with fluid, the tumour is termed a cystic adenoma (adenocele). The source of this fluid is of some interest, because adenomas are similar in structure to the gland in which they arise (Fig. 146), yet they are unable to 248 ADENOMA OF MAMMA 249 furnish the secretion pecuhar to the gland. In the case of adenomas growing from mucous membrane — e.g. the rectal and uterine adenomas — the glandular pits furnish a per- verted secretion. In the case of the thyroid gland, the adenoma is so en- capsuled that the secretion furnished by the gland-tissue of the tumour cannot escape, and, slowly accumulating, converts a^a"=S^' Fig. 146. — Section of an adenoma from a cMld's rectum. {Highly magnified.) the adenoma into a cyst. This occurs also in the mamma ; but it will be shown in connexion with adenomas of this gland that the fluid sometimes escapes by the natural duct. Adenomas may arise at any point in the mucous mem- brane of the gastro-intestinal tract, and they do not, as a rule, attain big dimensions. The adenoma that Lexer removed from the stomach of an adult, which was as big as a child's head, is very exceptional ; it grew, by a stalk as thick as two fingers, from the gastric mucous membrane near the pylorus. Adenomas exhibit pecuhar characters, and occasion dis- turbances which vary with the gland in which they arise; 250 EPITHELIAL TUMOURS it will therefore be convenient to consider each variety separately. It will be useful to point out that although adenoma and carcinoma may, and often do, co-exist in the same gland, an adenoma never becomes transformed into cancer. The best-known instance of the combination of an adenoma and carcinoma in the same gland was observed by Hutchinson. Carcinoma. Adenoma. Fig. 147. — Mamma in section ; it contains a fibro-adenoma surrounded by cancer. {3Iuseum, Royal College of Surgeons. ) In this instance the adenoma was embedded in a mammary carcinoma (Fig, 147), and the patient, a woman 46 years of age, had been aware of the existence of a tumour for twenty years. Adenoma of the mamma. — There are two varieties of mammary adenoma : 1, fibro-adenoma ; 2, cystic adenoma. 1. Fibro-adenomas. — These occur as spherical or oval tumours furnished with capsules, lodged in the superficial parts of mammae ; exceptionally they may be situated deeply in the breast substance. As a rule, they are firm and elastic Adenoma of mamma 2hl to the touch, and shp about under the examining finger. It is not rare to find a fibro-adenoma in each mamma, nor is it unusual to find more than one tumour in the same gland. When occupying a superficial position they will, even when small, project the skin so as to cause an irregu- larity in the contour of the breast ; very exceptionally they may be pedunculated. Although the majority of mam- mary adenomas do not exceed the dimensions of a walnut or of a Tangerine orange, some are as big as coco-nuts. Fig. 148. — Cystic adenoma with a glandular process. The cyst communicated with a duct in the nipple. Structurally they consist of fibrous tissue in which glandular acini are embedded ; the tumour itself is isolated from the surrounding gland-tissue by a definite capsule. Tumours of this character are commonly met with in the years succeeding puberty. It is rare to meet with them before the age of 15, but Patteson has published a care- ful description of two cases met with in girls of 13 years. These are probably the youngest cases yet recorded. The great rarity of fibro-adenomas of the breast before puberty is due to the simple construction of the breast in 252 EPITHELIAL TUMOURS the non-pubic girl. The gland-elements are represented by epithelium-lined tubes, which branch slightly, embedded in fibrous tissue. After puberty the gland-elements multiply, and this activity is accompanied by a corresponding active growth of the fibrous tissue in the breast. 2. Cystic adenomas. — As women increase in age, and especially if the breast has an opportunity of fulfilling* its function, then adenomas which arise in the gland contain Fig. 149. — Dilated galactophorous duct with intracystic growth. much more epithelium and far less connective tissue. The epithelial cells are larger, and approach in character those of the active mamma. Adenomas of this kind form far larger tumours than those to which the term fibro-adenoma is usually applied. Occasionally the glandular acini become dilated with fluid and form cystic spaces ; the tumour is then termed a cystic adenoma (or an adenocele). At times a cyst of this kind will retain its communication with the galactophorous duct (Fig. 148), and the secretion will sometimes escape at the nipple. Indeed, it is possible, when examining a breast, ADENOMA OF MAMMA 253 b"y gently squeezing the tumour to force a jet of fluid through the nipple. This is a diagnostic sign of great value. It sometimes happens after removal of a large cyst of this kind that a bristle can be passed from the cyst along a galactophorous duct. In some adenomas the cystic por- tion largely preponderates, the glandular element merely pro- jecting as a bud into the cyst. A sha,rp distinction, however, must be drawn between a cystic adenoma and a dilatation of a galactophorous duct with intracystic growth (Fig. 149). This variety is closely allied to duct-cancer and duct-papil- loma. Cystic dilatation of a galactophorous duct during lactation is known as a galactocele. Some of the rarer but larger and more formidable kinds of mammary adenomas are those which combine all the characters of the preceding varieties. That is, they con- tain much fibrous tissue, and numerous and fairly large cystic spaces, many of which are also almost completely occupied by intracystic processes. Mammary tumours of this kind sometimes attain very large proportions, weighing upwards of five or even ten pounds. These tumours have received a variety of denominations, such as sero-cystic tumours, adeno- sarcomas, and so on. However, clinically they are quite in- nocent, and do not recur after removal. It is a remarkable thing to remove a large complex ade- noma of this kind and to find it completely encapsuled, whilst the breast lies like a small process quite isolated from the tumour. The description of adenoma of other organs will be found in the succeeding chapters. Hutchinson, Sir J., " Adenoma Mamm£e, Supervention of Scirrhus." — Trans. Path. Soc, xxxix. 319. Lexer, Lelirhucli der allgemeinen Chir., 1905, ii. 371. Patteson, "Adenoma of the Breast in Childhood." — Journ. of Anat. and Phys., 1892, xxvi.. 509. CHAPTER XXV CARCINOMA (CANCER) This term, in the strict sense in which it is used by patholo- gists, signifies a malignant tumour arising in epithelium. The disease is of very great importance on account of its insidious onset, and, in the earliest stages, painlessness ; its progressive and irresistible destructiveness ; the manner in which it infects lymph-glands ; the extraordinary effects produced in different organs on account of the dissemination of the growth in the form of secondary nodules; the helpless- ness, misery, and pain it produces when fully advanced ; and the inability of medical and surgical art to deal effectively with it, save in the earliest stages. Although this disease was recognized in the dawn of medicine, we not only remain ignorant of its cause, but, in many instances, the diagnosis of the malady is uncertain in the living. This is not due to supineness on the part of investigators, but to the absence of what is called " specific symptomatology." Varieties of cancer. — Epithelium plays two parts in the animal economy : protective, as on the skin ; and secretory, where it is found in glands. When carcinoma arises from a surface covered with epithelium of the protective variety it is called squamous-celled cancer ; and when it arises in the epithelium of glands it is termed glandular cancer. The microscopic structure of a carcinoma is very simple and consists of columns of cells, so that when the columns are cut at right angles the section has the appearance of a number of alveolar spaces filled with epithelium. The walls of these alveoh are composed of fibrous tissue, presenting various degrees of density, in which blood- and lymph-vessels ramify. The cell- columns are not always simple, but may branch in various directions, and thus produce in some sections very complicated appear- 254 8QUAM0US-GELLED GANGER 255 ances, the softness or hardness of the cancer depending on the amount of fibrous tissue between the cokimns of cells. This plan of structure underlies all the varieties of malig- nant epithelial tumours, even those which arise on surfaces covered with squamous epithelium. The cells composing the columns depend upon the character of the epithelium in which the cancer originates, and this feature is so striking that the histologist can often pronounce with certainty the particular gland in which a cancer arose, merely from study- ing a carefully prepared specimen under the microscope. Stroma and parenchyma. — Every tumour, whether it be innocent or malignant, except the chorion-epithelioma (Chap. XL.), presents a stroma and a parenchyma. These two ele- ments are particularly observable in adenomas and carcinomas on account of the striking difference in the characters of the connective tissue and the epithelium. In the case of carci- noma, as the epithelial cells multiply and intrude into the adjacent tissue, the intrusion is answered by a formation of tibrous tissue: this response is less marked in the rapidly growing tumours than in those which grow slowly. This response of the tissues to irritation has been termed the specific tissue-reaction, but it is as obvious in many of the common forms of tissue-irritants, such as micro-organisms, and especially foreign bodies. Some of the most striking examples of the formation of fibrous-tissue capsules in response to irritation are those which form around an echi- nococcus-cyst lodged in the great omentum. In the case of a sarcoma a kind of investment is furnished for each cell, but in a carcinoma the cells are invested in groups pro- ducing in reality a fibrous-tissue maze. Squamous-celled cancer. — This may arise on any surface covered with stratified epithelium, but it is more common in situations where there is a transition from one kind of epithelium to another, and especially where skin and mucous membrane come in relation — e.g. the anus, vulva, or lip. It may make its appearance as a wart-like growth, more frequently as a small circular ulcer with raised ram- part-like edges, or as a fissure, and it is particularly apt to arise on the scrotum of the chimney-sweep (Chap. xxxv.). Sweep's cancer usually begins as a wart which is 256 EPITHELIAL TUMOURS familiarly known as a " soot - wart." A similar form of cancer is described as arising in men who work in tar, paraffin, and pitch. This matter has been investigated by Legge. Although the three clinical varieties of sqiiamous-celled cancer look so different, they are identical in structure. When sections are cut so as to include the margin of the ulcer and underlying tissue, the surface-epithelium will be seen invading it in the form of long, simple, or ramified columns. When the cones grow rapidly, the cells become flat- tened, and some finally cornify. In this way the so-called epithelial pearls or nests are produced. When lateral pressure is made on a fresh specimen, whitish plugs are forced out; these plugs are the cellular cones. It is important to bear in mind that the three clinical varieties of squamous - celled cancer occur in most of the situations liable to this disease, such as the lips, tongue, cheeks, vulva, and glans penis. This disease is occasionally met with in the urethra, the pinna, and in the conjunctiva, especially when it has been injured by lime. Examples of squamous-celled cancer of the pinna have been described by Hulke, Bowlby, and Williams. Cancers in the scars of burns. — It has long been known that chronic ulcers of all kinds are liable to become the seat of cancer. This is true also of the scars left by burns, and especially of the chronic ulcers so common along the edges of the scar left by an extensive burn. An interesting contribution to this matter we owe to Neve. During twenty years 4,902 tumours have been removed at the Kashmir Mission Hospital. Among this number 1,720 were malignant ; 1,189 of the malignant tumours Avere classed as carcinomatous, of which 848 arose on thighs or abdomen. The cancer arises in ulcers caused by Fig. 150. — Section of an epithe Hal cone. {Magnified.') GANGER 257 burns from the use of the kangri, a portable fire-basket. The kangri is suspended round the waist under the flowing robes of the natives of the cold hills of Kashmir (-Fig. 151). In many instances the carcinoma arises in scar-tissue. The use of the kangri by the Kashmiri is akin to that of the chauf- ferette by poor old folk in France (p. 246). X-ray cancer. — Since the X-rays have been employed for the detection and cure of certain diseases, it has been Fig. 151. — A group of Kashmii'i with the portable fire-baskets known as kangri. {After E. F. Neve, Brit. Med. Journ., 1910, ii. 589.) discovered that they sometimes produce changes in the skin known as X-ray burns, or X-ray dermatitis, which are extremely difficult to treat and in some instances become cancerous. The alterations in the skin covered by the term X-ray dermatitis may affect not only those who receive the rays designedly for the cure of local disease but also those who apply them, and the latter appear to be the greater sufferers. The earliest changes consist of an erythema around the base of the nails, which become brittle and degenerate into shapeless masses. The skin reddens and small warts appear ; 11 258 EPITHELIAL TUMOURS cracks and ulcerated patches occur and refuse to heal. The ulcers and cracks are, extremely painful, and in a small proportion of cases become malignant. Whilst these changes are progressing in the skin, the deeper tissues undergo nutritional changes and the bones of the fingers waste. Eowntree, who has had opportunities of studying the path- ology of several cases of X-ray cancer, states that the growth has all the typical features of squamous-celled carcinoma, cell-nest formation being well marked. The precancerous stage — the stage of chronic dermatitis — is prolonged, and the transition to carcinoma is effected by slow and insensible gradations. X-ray cancer is of low malignancy and only occasionally infects the lymph-nodes ; metastasis is unusual. The only available treatment is amputation of the affected fingers, or the hand. Recurrence is unusual. Rowntree states that since the introduction of X-ray treatment for lupoid ulcers the percentage of cases in which such ulcers have become carcinomatous has materially in- creased. Local changes which clinical observation has shown to precede cancer are termed precancerous conditions. A squamous-celled cancer, Avhen left to follow its own course, may extend and involve extensive tracts of tissue, or fungate and form huge granulating dendritic masses. In both cases the superficial parts are continually cast off in a foul, fetid discharge containing sloughs of tissue, cellular detritus, and blood. Vascular tissues, such as skin, muscle, and mucous membrane, are quickly infiltrated and destroyed ; even bone is rapidly eroded. Cartilage resists invasion ; this is seen in a striking way in those rare instances in which cancer attacks the pinna ; the skin and soft tissues quicldy disappear, whilst its cartilaginous framework stands promi- nently out amidst the surrounding ruin. In whatever situation squamous-celled cancer occurs, it destroys life rapidly. The quickness with which it ulcerates and overcomes all resistance enables it to open large blood- vessels should any lie in its way ; hence death from haemor- rhage is frequent. When the cancer is near the air-passages, oul material is inspired and initiates septic pneumonia. GLAND-GANGEB 259 Gland-cancer. — This variety arises in the epithelium of secreting glands ; it is exceedingly common in some and rare in others, so it will be convenient to discuss the liability of the various glands separately ; but the general features of this disease are the same in whatever gland it arises. A striking feature of cancer is the fact that it does not form a circumscribed tumour. When examined clinically it is rarely possible to define the limits between the tumour and the surrounding tissues, and this indefiniteness is more obvious when, in the course of an operation, the surgeon cuts into it ; but, what is more significant, when the peri- phery of a cancer is subjected to microscopic scrutiny the eye of a competent pathological histologist is unable to discern the precise limitation of the cancerous territory. This illimitation of cancer constitutes one of the greatest obstacles in dealing with it surgically ; for if with the aid of a microscope there is difficulty in defining its limits, how uncertain the surgeon must be in determining its extent with only fingers and eyes to guide him during an operation ! This has led to the practice, in recent years, of complete ex- tirpation, whenever possible, of cancerous organs. Although a cancer is for a time limited to the gland in which it arises, we have no means of distinguishing with any reasonable certainty, when the individual comes under observation, that the cancer is limited to the gland, for its outrunners quickly involve surrounding structures, whether skin, fat, mucous membrane, muscle, or bone. When adjacent parts are infiltrated or permeated in this way, it is convenient to describe them as being implicated in the cancer. This implication of organs is a grave feature, and a common cause of death, and it is often a bar to operative intervention. The insidious way in which fascia is permeated by carcinoma has recently been made the subject of a careful investigation by Handley (see p. 269). Although cancers, like all epithelial structures, are in free communication with the lymph-system, they are poorly supplied with blood : this leads to retrograde changes, which it is customary to describe as degeneration. The commonest of these is known as colloid degeneration, in which the epi- thelium in the cell-columns becomes changed into a structure 260 EPITHELIAL TUMOURS less material resembling jelly: this change is particularly common in cancer of the stomach and colon. It is well known that a primary cancerous lesion may undergo retro- gressive changes and almost disappear. The variety known as " withering cancer " or " atrophic cancer " of the breast is an example of this. Patients with this kind of cancer have lived ten, fifteen, and even twenty years. The not un- common form of cancer found in the colon, especially in the sigmoid flexure, where the growth encircles and narrows the gut so tightly that it seems as if a piece of cord were tied around it, is really a primary carcinoma undergoing sponta- neous cure; but it surely destroys life, if not from its mechanical effect in obstructing the colon, by infecting the liver and peritoneum. Thus cancer manifests itself differently in the same organ, and its effects vary more widely in diverse organs. For example, primary cancer of the liver is always massive, and leads sometimes to enormous enlargement of this organ. This is also true of secondary deposits in the liver, for they attain a greater size in its tissues than elsewhere. Hillier suggests that, in addition to the large size of the liver, its small proportion of connective tissue, its blood-supply, copious • and rich with food products, may explain this ; carcinomatous growths contain a large amount of glycogen, and its presence in the hepatic cells may have something to do with the way in which cancer flourishes in the liver. Secondary (or im- plantation) cancer of the ovary sometimes forms masses as big as a man's head. This exuberant growth probably depends on the abundant blood-supply of the ovary and the exclusion of pathogenic micro-organisms. Primary cancer of the pancreas seldom forms a large mass, and usually appears in the head of the gland as an ill-defined swelling. The difference in the proportion of fibrous tissue in the liver and in the pancreas may offer some explanation of the variation of size in cancerous masses in the two organs. Infection of lymph-glands. — The surfaces of our bodies, whether skin or mucous membrane, are rich in lymphatics, and as the secreting glands are primarily derived from these surfaces, it naturally comes about that they are in free com- LYMPH- GLAND INFECTION 261 munication with the lymphatics and lymph-glands or lymph- nodes. The lymphatics involved in the cancerous material convey the cancer elements to the lymph-glands, and these may become so surcharged as to burst their capsules. Lymph- glands enlarged in this way sometimes form very con- siderable masses, and it is not uncommon to find a primary carcinoma with a diameter of 2 cm. associated with a collection of lymph-glands as big as a fist. Lymph-gland infection varies in rapidity and degree ; great differences occur not only in regard to cancer of particular organs, but also in relation to the same organ in different individuals. Sometimes lymphatic channels are so stuffed with cancer- ous material that they may be dissected from the connective tissue and traced to the lymph-gland. When cancer arises in lupus-scars the adjacent lymph- nodes are unaffected. This is attributed to the previous destruction of the lymphatics by the lupus (Wild). Occasionally the ducts from the lymph-glands about the receptaculum chyli, the receptaculum itself, and the thoracic duct are stuffed with cancerous material (Fig. 152). The relation of the growth to the wall of the duct shows that the implication of its structures is complete ; it is not due to the mere blocking of its lumen with cancerous tissue, resembling the clot in a thrombosed vein, but its walls are infiltrated with the cancerous tissue in the way that sarco- matous tumours implicate the walls of large veins. Obstruction of the thoracic duct by extension of cancer has been noticed in association with primary cancer of the stomach, uterus, rectum ; and careful descriptions of the con- ditions have been published by Unger, Weigert, Troisier, and Hillier, among others. Perhaps the most remarkable feature of the complication is the absence of any indication that this duct was obstructed, and in no case was chylous ascites observed. The implication of the thoracic duct in cancer of the stomach explains the enlargement of the lymph-glands at the root of the neck ; this is a sign of diagnostic value. The extent to which lymphatic infection has occurred is a matter which cannot be accurately defined in a given case of carcinoma, and this adds an additional factor of uncertainty in estimating the results and value of surgical ^ ll"l,'l'jiill ^ Fig. 152. — Thoracic duct and receptaculum, with some adjacent glands, stuffed with cancerous material secondary to cancer of the rectum. {Sillier.) 262 DISSEMINATION 263 procedure. Lymph-gland infection is always an element of danger. When the cervical glands are enlarged they inter- fere with the trachea and oesophagus ; they also become firmly adherent to the sheaths of big vessels, and, as the glands break down, the ulcer opens up the jugular vein, or the carotid artery, while, in the inguinal region, the femoral vessels are eroded. Lymph - glands, when enlarged and stuffed with carcinomatous cells, have a great tendency to soften in the centre and form spurious cysts. When the skin becomes implicated, extensive portions of the infected glands slough, and leave large, horrible holes, from which a fetid fluid issues, whilst the edges of the chasm produced by the sloughing continue to extend and involve the neighbouring tissues. The size of the cancerous mass, produced by the infected lymph-glands and the tissue infected by them when they become so stuffed with cancer that they burst their capsules, is often, as has already been mentioned, out of all proportion to the initial lesion ; indeed, in many instances the patients are little troubled by the primary ulcer, which may be so small and inconspicuous as to escape observation until the enlargement of the lymph-glands compels them to seek advice, which leads to a search for the primary lesion. It is not uncommon when this focus is situated in a recess in the mouth or pharynx for the cancerous ulcer to be so small as to be completely overlooked, and then the cancerous gland- mass in the neck is supposed to arise in epithelial vestiges of the branchial folds. It is also possible that the primary focus undergoes retrogressive changes and heals spontaneously, while the gland-infection proceeds to the patient's destruction. Dissemination. — Cancers are extremely prone to dissemi- nation, which means the formation of secondary growths resulting from the deportation of minute fragments of cancer (cancer emboli), which may lodge in any organ or tissue. The cells which give rise to secondary nodules are transported by lymph- and blood-vessels, and by an insidious process known as permeation. When these minute emboli and cancer particles lodge in suitable positions they multiply, giving rise to a growth which in its histologic features exactly resembles the parent tumour. So faithful is this reproduction that the 264 EPITHELIAL TUMOURS nature of the primary tumour can often be correctly interred from a microscopic examination of a secondary nodule. The amount of dissemination varies greatly. In some cases secondar}^ deposits will be found only in the liver, whilst in another and apparently identical case, in so far as the structure of the tumour is concerned, secondary knots occur in almost every organ of the bodj^ including the skeleton. In the case of squamous-celled cancer it cannot be said that secondary deposits are rare, but dissemination certainly happens far less frequently, and never so extensively as in cancer arising in secreting glands. It is also noteworthy that the squamous-celled variety is in some situations more liable to disseminate than in others. For example, secondary deposits are rarely met with when this disease attacks the larynx, or the mucous membrane in relation with the mandible or maxill&e, or the oesophagus. The explanation sometimes offered of this peculiarity is that carcinoma in these situations usually runs a rapid course, and often destroys life so quickly that the period is too short to allow of the formation of secondary nodules. This is inadmissible, for in cancer of the scrotum dissemination is almost as exceptional as when the larjmx is attacked. Secondary deposits of cancers are not always so small as merely to merit the name of knots, but form occasionally tumours of some magnitude. The vitality and power of independent growth possessed by cancer emboli is very remarkable. These minute epithelial emigrants not only live and grow, but reproduce the pecu- liarity of the primary cancer. It is astonishing to find a secondary cancerous deposit in the humerus with all the characters of the s^lands of the rectum : a multitude of secondary nodules in the skin with the structural features of gastric glands ; nodules in the lungs exactly reproducing that peculiar form of hepatic carcinoma which arises in the biliary ducts ; the familiar closed follicles of the thyroid gland repro- duced in the body or spinous process of a vertebra ; nodules resembling the structure of mammary carcinoma in the ovary, brain, or choroid coat of the eye ; and a mass growing from the frontal bone with all the characters of the prostate gland, secondary to cancer of that organ. It is one of the great BIS SEMINATION 265 triumphs of pathological histology that it has demonstrated that carcinoma takes its type of epithelium from the secreting gland in which it arises. This power of independent growth possessed by the epithe- lium of cancer is a very dangerous feature, and does not always need blood- or lymph- vessels for its manifestation. It X 130 Fig. 153. — Section through a portion of an inguinal lymph-gland infected with cancer : the primary disease was in the rectum. (After Foulerfon. ) sometimes happens that an abdominal viscus is attacked by cancer, and a small outgrowth makes its way through the peritoneal covering and bursts, and sheds its cells into the general peritoneal cavity ; these are distributed by the peri- toneal fluid and the movements of the bowels, and in a few weeks the whole of the serous membrane will be dotted with hundreds, and sometimes thousands, of nodules, each repro- ducing the type of the parent tumour. This mode of epithe- lial infection of the peritoneum I have found in cancer of the gall-bladder, ovary, and especially in cancer of the body of the uterus; no form of dissemination gives rise to such 266 EPITHELIAL TUMOURS innumerable secondary nodules as this, nor demonstrates in a more remarkable way the power of epithelium to engraft itself, to live, and to grow. All cancer-cells which fall on the peritoneum do not live in spite of their vitality, for this membrane has the power of destroying them. Many which grow are hindered from doing much harm, for they become encysted. Implanted cancer flourishes best on connective tissue {see Cancer Infection, p. 270). Secondary deposits of cancer may occur in any organ and tissue of the body; my own observations teach me that among the malignant epithelial tumours, cancers of the breast and of the pylorus give rise to the wadest form of dissemina- tion. Thus cancer is very infectious to the individual affected with cancer, but not to others. The rarest of all tissues in which to find secondary deposits is voluntary muscle, and the rarest of all organs is the heart. Secondary deposits are even met with in the eyeball, and it is a curious fact that the great majority of cases occur in association with mammary cancer, and in one exceptional case both eyeballs con- tained secondary nodules. Secondary deposits in the S'lobe have also been observed in connexion with cancer of the stomach and thyroid gland. (Rowan and Devereux Marshall. ) Secondary cancer of the lung. — The extraordinary frequency with which the lung is infected with malignant disease, whether sarcoma or carcinoma, is due to the circulation. In the case of sarcoma the particles gain the circulation entirely through the veins ; but in carcinoma, while the cancer emboli also enter the blood-stream by the veins, the most usual channel is the right lymphatic or the tho- racic duct, according to the situation of the primary focus of disease. In this event, of course, the migratory elements are discharged into the innominate veins, carried thence into the pulmonary vessels, become filtered from the blood by the capillaries of the lungs, and, after their arrest, find in this vascular tissue an excellent soil in which to grow. In discussing secondary deposits in the lungs due to cancer-emboli, it must not be forgotten that cancer of adja- cent organs, such as the mammary glands, the oesophagus, stomach, etc., may locally invade the pleura (permeation), DISSEMINATION 267 and give rise to a widely scattered crop of miliary nodules on the pulmonary pleura. This mode of infection must be distinguished from that in which the lungs are infected by emboli transported by the blood. Much new light has been thrown on the way in which cancer implicates the chest- wall and infects the thoracic as well as the abdominal organs, by the researches of Handley, "5S^^^^^-"' '- f Fig. 154. — Canceroas embolus iu a pulmonaiy capillary embedded in a thrombus. {After Schmidt.) who has especially studied the manner in which cancer-cells slowly creep along the planes of fascia. There are many points connected with the dissemination or generalization of cancer which are not clearly explained. As we shall find later on, there are two views as to the manner in which secondary cancer forms in bone, namely, the embolic theory and the permeation theory (Handley). Cancerous cells enter the blood-stream by implicating veins, or by the lymphatics. M. B. Schmidt has shown that these cells excite thrombosis, and the thrombus or clot contracts upon and may ultimately destroy them (Figs. 154 and 155). 268 EPITHELIAL TUMOURS This defending or prophylactic power of the blood prevents colonization of the blood-stream. In regard to secondary cancer of vascular organs, sucb as the liver, bones, and ovaries, reference may again be made to its massiveness as compared with the size of the primary focus ; in these circumstances, indeed, the continual progress of the disease may be described as " ceaseless cell-proliferation." These large secondary formations are instructive from another point of view : primary cancer always arises on a surface to which air or intestinal gases have access, and Fig. 155.- — Pulmonary capillary in section, showing cancer emboli in its lumen. {After Schmidt.) therefore pathogenic micro-organisms. The result is ulcera- tion, sepsis, and destruction of the growth, followed by septic infection and its deleterious consequences. Secondary deposits in the liver, ovaries, and bones are not so exposed in their early stages to local infection, and thus grow undisturbed until they attain proportions suffi- cient to cause ulceration of the skin, or involve the bowel and become infected ; then death quickly follows. As a matter of fact, cancer is a very chronic disease, save for accidental infections, and, as in such chronic diseases as tabes and the various sclerotic changes of nerves, blood- vessels, kidneys, and liver, death really ensues from a group of diseases known as terminal infections, such as uraemia, pneumonia, peritonitis, meningitis, and the like, due to the activity of many sjsecies of pathogenic micro-organisms. PERMEATION 269 Secondary deposits of cancer in bone. — The distribution of metastatic cancer in bone has been made the subject of careful observation by Recklinghausen, Theile, Cone, and others. In the preceding section some reference was made to this phenomenon. The chief sources of cancer deposits in bone are primary cancer of the prostate, thyroid, and mammary glands : they also occur in connexion with primary cancer of the stomach, oesophagus, uterus, and rectum. Prostatic cancer shows an especial tendency to disseminate in bone, and Recklinghausen points out that the cancer-cells lodge in the vascular channels of the marrow and form a de- posit; as this grows, outrunners make their way through the adjacent foramina of the bone and form subperiosteal deposits. A careful examination of the distribution of secondary cancer- ous deposits in bone bears this out, for they occur in greatest number where the foramina of bones are largest and most numerous, and a critical inspection of bones invaded by secondary cancer also shows that in many bones osseous tissue exists between the medullary cavity and the periosteum, so that the growth has not simply made its way through by erosion. The effect of secondary deposits growing in bone is ot three kinds : — When growing slowly it may simply erode the osseous tissue, or may cause great expansion of the bone accompanied by osteoplastic changes ; or there is marked infiltration of the bone without expansion, but with osteoplastic changes. It has been suggested that the osteoplastic changes are due to chronic venous congestion on account of the multipli- cation of cancerous cells acting as a thrombus. These observations seem to show that subperiosteal can- cerous deposits are due to extension of intramedullary deposits through the foramina to the subperiosteal tissues, and are not primarily subperiosteal. The matter, however, admits of another interpretation. Handley has made a very careful investigation of the mode in which cancer of the mamma dis- seminates, and shows that it spreads in the thoracic wall by permeation, a slow, progressive, centrifugal serpiginous process, which is an actual growth of the cancer along one or 270 EPITHELIAL TUMOUHS other lines of the parietal layers in continuity with the primary groAvth. He has carefully analysed the situation of secondary deposits in this disease, and points out that they appear in the near neighbourhood of the primary focus, and as the disease advances the nodules occur at greater distances from the primary focus until at last, if death is unduly delayed, they appear in the trunk ends of the limbs. Hence the distal halves of the limbs enjoy an almost invari- able immunity from the cancerous nodules. This applies to the bones of the limbs as well as to the skin. He believes these facts to indicate that the superficial spread of cancer takes place by permeation of the deep fascia. Moreover, he has carefully studied and traced this infiltration of the deep fascia microscopically. Handley believes that visceral deposits of mammary can- cer do not arise from cells conveyed by the blood : according to his researches they occur through the fine anastomotic lymphatics w^hich pierce the parietes and infect the subserous lymphatics of the pleura and peritoneum. Cancer- cells then escape into the thoracic and abdominal cavities, implant themselves on the surface of the viscera, and there give rise to deposits which terminate the life of the patient {see Chap. li.). Cancer-infection. — It has long been known that normal cutaneous epithelium, when accidentally engrafted into sub- cutaneous tissue, the cornea, or the iris, will live and grow. It has also been demonstrated beyond all cavil that when women have been ovariotomized, especially in cases of large ovarian adenomas, tumours have in some instances grown in the abdominal cicatrix; these on microscopic examina- tion have displayed cysts furnished with the regular large mucin-bearing cells so characteristic of some varieties of ovarian tumours. As these tumours in the cicatrix have been unassociated with any recurrence in the pelvis, or with secondary nodules in the peritoneum or in the viscera, the conclusion is irresistible that they were due to infection of the edges of the abdominal incision in the course of the ovariotomj^ These cases are profoundly interesting, be- cause they illustrate what often happens in the course of an operation for the removal of a cancer; and it is this GANGEB-INFEGTION 271 local soiling of the wound with minute cancerous particles that constitutes the accident which I have called cancer- infection. A careful study of the clinical aspects of cancer, as well as the most critical inquiry in the post-mortem room, has convinced many that cancer exhibits peculiarities in regard to mode of growth, infection of lymph-glands, dissemination, and the way it destroys life, according to the gland in which it arises. Even this only partly expresses the real truth, for not only is the course of carcinoma of the same gland widely modified by age and constitution, but the same disease in two patients, apparently alike in age and environment, will progress so differently that no surgeon can predict with any reasonable certainty the expectancy of life, result of operation, liability to dissemination, or the chances of recurrence. Therefore, in deciding whether it will be to the patient's advantage to have a cancerous organ extirpated, the surgeon is guided by the known peculiarities of the particular organ affected, the extent to which the adjacent tissues are impli- cated, the degree to which the associated lymph-glands are infected, and the absence of signs indicating dissemination. In spite of every care, the o]3eration is occasionally followed by such rapid local recurrence that the course of the disease is accelerated rather than retarded. It is a fact which every surgeon who has had much experience in operating for cancer must have noticed, that, in some instances where he has conducted carefully planned but extensive operations for cancer, the patient has had rapid recurrence, and the disease has manifested itself in a manner far worse than when left to run its natural course. This phenomenon, I believe, may be explained. In removing the affected organ the infected lymphatics and blood-vessels stuffed with the cancerous material are divided, and the cancer-cells are let loose over the damaged tissues, wdiich they infect, and lead to an extensive outbreak of local cancer. Knowledge of this kind is important, because it leads us to exercise greater care in keeping well wide of the diseased area whilst removing it; and though we cut out the cancer with its implicated lymphatic ducts and infected lymph-nodes, we should exercise every precaution not to incise the diseased 272 EPITHELIAL TUMOURS parts and thus unwittingly scatter the diseased cells over the denuded surfaces. I have more than once seen patients who had been sub- mitted to operation for mammary cancer, and in whom the removal had been imperfectly carried out, present on both sides of the scar a series of cancer nodules at each stitch- hole, due to infection by the needle and thread in the course of the operation. The most striking example of cancer- infection under my own observation occurred in a woman aged 58 years. I excised a cancer from her descending colon. Nine months later a tumour as big as a bantam's egg was removed from the scar left by the incision in the belly-wall : this tumour exhibited microscopic features identical with those of the primary tumour in the bowel. In the course of the operation I carefully inspected the interior of the abdomen and found the omentum and bowels free from all visible signs of dissemination. It is obvious that the edges of the abdominal incision were infected with cancer during the removal of the primary tumour. Transference of cancer by contact. — Many cases have been reported which are supposed to prove that cancer may be transplanted by the direct contact of a cancerous surface either with another part of the infected person's body or with another person. The examples of the first condition usually mentioned are the infection of the skin of the arm from contact with an ulcerating carcinoma of the breast, and the infection of a labium by a squamous-celled cancer in the opposite labium. I have never seen the upper lip infected from contact with a cancerous lower lip, nor the cheek infected save by extension of the growth in the case of cancer of the tongue. It is also a matter of common observation that even in extensive cancer of the tongue, jaws, or pharynx, quantities of cancerous particles find tbeir way into the stomach, but the mucous membrane of the gastro-intestinal tract escapes. Surgeons who are actively engaged almost daily in performing operations for cancer frequently cut or prick their fingers, but a cancer transplanted in this way is unknown ; in contrast to this, it may be mentioned that there is probably no surgeon who has not infected himself in this way with some form of septic disease. EEBDDITY 273 This should make us careful in accepting evidence in regard to what is sometimes called cancer-d-deux, in which a cancerous ulcer appears on the penis of a man cohabiting with a woman suffering from cancer of the neck of the uterus, or vice versa. Heredity. — This is another difficult problem, or it would be better termed a vexed question, in regard to cancer and malignant disease generally, because so much that appears to be affirmative is founded on false facts — that is, on cir- cumstances that cannot be tested or proved. The statement that the father died of cancer of the prostate, and the mother of a sarcoma of the humerus, is scarcely a good explanation of the cause of a malignant dermoid or em- bryoma in their infant daughter. When several female members of a family die from cancer of the breast, it will be found, on careful inquiry, that they have lived in the sauie environment. The question of cancerous inheritance bristles with difficulties, many of which are at present insuperable. Bayha, H., " Ueber Lupuscarcinom."— -Sei^. z. Idin. Chir., 1888, iii. 1. Berry, James, " Carcinoma following Lupus of Face." — Trans. Path. Soc, 1891, xlii. 308. Bowlby, Anthony A., "A Case of Epithelioma of the Ear."— lV««s. Path. Soc, 1884, XXXV. 330. Brand, " The Etiology of Cancer."— 2?ri/. Med. Journ., 1902, ii. 238. Cone, " A Case of Carcinoma Metastasis in Bone from a Primary Tumour of the Prostate." — Bull. Johns Hopldns Hos^p., 1898, xv. 114. Foulerton, "A Case of Squamous- celled Carcinoma of the Finger, associated with frequent and prolonged exposure to X-rays." — Trans. Path. Soc, Iviii. 327. Handley, W. Sampson, " The Centrifugal Spread of Mammary Carcinoma in the Parietes."— J.w7t. of Middx. Hosp., 1904, iii. 27. Hillier, W. T., "Carcinoma of the Thoracic Duct." — Tra7is. Path. Soc, 1903, liv. 153. Hillier, W. T., "Some Remarks on Cancer of the Liver and Panci-eas. " — Arch, of Middx. Hosp., 1903, i. 123. Hulke, J. W., " Epithelioma of the Side of the Head perforating the Skull." — • Trans. Path. Soc, 1875, xxvi. 187. Langhans, D. G., " Primarer Krebs der Trachea und Bronchien." — Yirchow's Arch.f. path. Anat., liii. 470. je, T. M., " Pitch Cancer." — Brit. Med. Journ., 1910, ii. 1370. S 274 EPITHELIAL TUMOURS Marshall, C. L. Devereux, "Metastatic Carcinoma of the Ej'eball." — Hoy. Lond. Opltthal. Hosp. Bepts., 1897, xiv. 415. Mathieu, Albert, et Hattan-Lorrier, L., " Cancer du Canal Tlioracique, con- secutif a un Cancer de I'Estomac." — Bull, et Mem. de la Soc. Med., Paris, 1898, XV. 827. Neve, E. F., " One Cause of Cancer as illustrated by Epithelioma in Kashmir." — Brit. Med. Jowrn., 1910, ii. 589. Passler, Hans, " Ueber das Primare Carcinom der Lunge." — Virchow's Arcli.f. j,ath. Anat., cxiv. 191. Pryce-Jones, C, " The Cytology of the Blood in Malignant Disease, with Literature." — Hepts. from the Cancer Besearch Laboratories of Middx. Hasp., 1902, i. 113. Rowan, J., " Metastatic Carcinoma of the Choroid from a Primary Carcinoma of the h\mg."— Trans. OpUlial. Soc. of U.K., 1899, xix. 103. Rowntree, C. W., " Contribution to the Study of X-Ray Carcinoma and the Conditions which precede its Onset." — Arch, of Middx. Hosp., 1908, xiii. 182. Theile, " On Secondary Carcinomatosis of Bones and Osteoplastic Changes connected with them." — Trans. Path. Soc, Iviii. 814. Troisier, " Le Cancer du Canal Thoracique." — B^ill. et Mem. de la Soc. Med., Paris, 1898, xv. 455. Unger, E., "Krebs des Ductus thoracicus."— Virchow's ^rc/j./. ^;a^7i. Anat.,. 1896, cxlv. 581. Williams, W. Roger, " Epithelioma of the External Ear." — Trans. Path. Soc, 1884, XXXV. 331. CHAPTER XXVI CONCERNING THE CAUSE OF CANCER The cause (pathogenesis) of carcinoma has for many years been a fascinating subject of inquiry and has led to much speculation, some of which has had great influence in directing research along particular lines. Great obscurity surrounds the cause of this disease, because our knowledge depends on observation alone; all attempts to elucidate the problem by experiment have been complete failures, therefore observation has been supplemented by theory. Among the hypotheses or guesses at truth in connexion with this matter there are three which require consideration : — 1. The Embryonic. 2. The Parasitic. 3. The Biologic. 1. The embryonic theory. — Cohnheim attempted to ascribe the origin of malignant tumours to cells, or groups of cells, which are not utilized in the development of the body in its early or embryonic stages, and he assumed that these residues or " rests " retain potential powers of growth, and later in life they suddenly and without obvious provocation assume active growth and become tumours. This theory, unsupported by any concrete evidence, was advanced by Cohnheim as an explanation of the origin of connective-tissue and epithelial tumours. The great argu- ment against it was to the effect that unutilized embry- onic tissue or rests had not been demonstrated ; but it suggested a line of inquiry in which observation proved the existence of tissue-islets which in some instances could be regarded as potential sources of tumours belonging to the so-called innocent group. Experimental inquiry did not 275 ^7« EPITHELIAL TUMOURS support the theory, and as an explanation of the origm of malignant tumours it has signally failed. The term rests, used in discussmg the pathogenesis of tumours, should be reserved for detached fragments of secret- ing glands and isolated portions of epithelium. Examples of this kind occur in connexion with the spleen (splenculi) ; an accessary pancreas is well known, and it may be lodged in the wall of the duodenum or jejunum. Accessary thyroid glands and adrenals are by no means uncommon, and reference is made to them in appropriate places in this book. In addition to rests being represented by detached portions of an organ, it has been shown that they ma}^ occur as isolated portions of gland- tissue within the organ itself. Gland-islets of this kind have been observed in the liver and in the mamma; it is possible that such sequestered portions of glandular tissue may be the source of encapsuled adenomas. Rests composed of epithelium have been detected in the line of the mesopalatine suture, and on the gums {see p. 231) ; but in the non-epithelial tissues they do not admit of ready recog- nition. The best examples are the islets of cartilage in the vicinity of the epiphysial lines of long bones in rickety children (p. 26). Such belated pieces of cartilage maj^ be the source of a chondroma. Efibrts have been made to extend Cohnheim's theory in regard to rests as an explanation of the origin of malignant tumours by supposing that islets of glandular tissue may be formed in organs as a result of inflammatory changes, such isolated tissues being supposed to acquire proliferative power and become tumours. In order to distinguish belated tracts arising in this way from the embryonic residues, it has been proposed to term them " post-natal rests." This extension of the embryonic hypothesis has not met with success. Care must be taken not to confound rests with vestiges. The term vestige should be reserved for those organs which are of importance to the embryo and foetus, but useless to the adult, such as the vitello-intestinal duct, the round ligament of the liver, the mesonejihros, etc. ; also the repre- sentatives of those organs which, though utilized in the male, are useless in the female, and vice versa, such as Gartner's duct, the parovarium, etc. There are structures which, so far CAUSE OF CAXCER 277 as we know, serve no useful purpose in any vertebrate at present living, but were doubtless of importance to their ancestors. Examples of this are the central canal of the spinal cord, the cerebral ventricles, pineal eye, etc. Cohnheim's theory has commanded much attention: it is in itself a brilliant generalization, and has served a valu- able purpose in leading to a great extension of knowledge concernincj vestiges and rests. In regard to congenital defects of tissues as the subsequent sources of malignant tumours, the most obvious are those known as birth-marks or moles. Many hundreds of these blemishes come under the notice of trained observers yearly, but probably not one black mole in a thousand becomes the source of a melanoma, or an endothelioma. Trauma in relation to malignant tumours. — Injury as an etiological factor has only been seriously advanced in the case of the breast and the testicle, two organs particu- larly exposed to injury. The majority of women receive in the course of their lives an accidental blow upon their breasts, and the frequency with which women attribute the cause of a cancerous tmnour within the breast to an injury is largely due to the belief, deeply rooted in their minds, that such injuries are the common cause of cancer. About 10 per cent, of patients with this disease in their breast can, and do, assign a specific injury as the starting-point. Sarcomas are unusual tumours of the breast and form about 10 per cent, of the malignant tumours of this organ. Of some recent statistics the following may be mentioned : Among 335 tumours of the breast, 33 were sarcomas (Poulsen of Copenhagen). In Bergmann's clinic there were 34 sar- comas among 359 mammary tumours. Schmidt found in 139 cases of malignant disease of the breast 126 examples of carcinoma and 13 of sarcoma. Homer amoncr 172 malignant tumours of the breast found 14 sarcomas. Sar- coma occurs at an earher age than cancer, and women are most liable to this form of malignant disease between the twentieth and fortieth years of life, whereas cancer is most frequent between the thirty-fifth and fiftieth years. As with cancer, patients often attribute the tumour to 278 EPITHELIAL TUMOURS an injury, especially to what may be called an " intensive injury." It is a fact that surgeons see many patients with sarcoma and carcinoma who cannot recall any injury to the part : and of the enormous number of contusions and injuries only an infinitesimal proportion is followed by a malignant tumour. Small as is this number, the circumstances relating to these sequences are such as to lead surgeons to believe that a single " intensive " injury may occasionally induce the growth of a sarcomatous tu'niour, and place it outside the category of mere coincidence. The attitude of surgeons, as reflected in their writings, towards trauma, or physical insults, as an etiological factor in the production of malignant tumours in the breast, justifies the following statement : — In regard to cancer (carcinoma), all surgeons of experience admit that there is a definite history of intensive mechanical injur}/^ in about 10 per cent, of the patients. They are very careful not to express a definite opinion as to the causal relationship of such injuries to the formation of, cancer in the breast. There is also a paucity of published statements from surgeons of great experience affirming trauma as a cause of mammary cancer. In regard to sarcoma of the breast, there is a definite opinion held by experienced surgeons to the effect that there are many carefully observed and thoroughly reported cases in which primary sarcoma of the breast has quickly supervened on a single intensive injury. The sarcomatous nature of such tumours has been ascertained by a microscopic examination at the hands of a competent pathologist, and their malignant nature has been confirmed by the early death of the individual. It is undeniable that a single in- tensive blow or knock on the breast may be occasionally followed by a sarcomatous tumour. 2. The parasitic theory. — Many who are thoroughly acquainted with the clinical and pathological features of carcinoma feel strongly that this disease will ultimately come to be defined as a chronic infective disease due to a micro- parasite luhich selects an epithelial cell. The brilliant results of microscopic inquiry during the last CAUSE OF GANGER 279 thirty years into the causes of disease have added to the number of parasitic diseases previously known to us. It has become customary, in describing the vegetable and animal parasites infesting man, to speak of the flora and fauna of the human body. This application of a natural- history expression is useful, perhaps even picturesque, and it is certainly an improvement on many of the dry and commonplace terms used in medical writings ; moreover, the expression is true. As the living things in a brook thrive best in certain haunts, so the vegetable and animal forms which infest animal bodies exhibit a marked preference tor certain organs and tissues in which to live and grow. For example, the demodex prefers the hair-follicles, whilst ankylostomum selects the mucous mem- brane of the duodenum ; the malaria parasite finds its way into an erythrocyte ; filarise swim freely in the liquor sanguinis ; CoccidiuTYi ovifornie finds its way into the epithelium of the biliary passages ; and the embryo of Tcenia echinococciis prefers subserous areolar tissue, whilst the adult form of this tape- worm chooses the mucous membrane of the dog's duodenum. Among infectious diseases, the most extraordinary and some of the deadliest are those in which the infecting agent gains access to the body by inoculation, that is, through abrasions, cuts, or punctures of the skin or mucous membrane. Familiar examples of this are tetanus, hydrophobia, leprosy, glanders, actinomycosis, and syphilis. The point of inoculation is known as the primary focus of the disease, and at this source the parasites multiply, enter the circulation and lymph- stream, whence they may be distributed throughout the body, often to form secondary foci of disease which interfere with the functions of the organs in which they may chance to grow, as well as with the nutrition of the body by means of the toxins they brew and discharge into the blood, producing a form of slow poisoning. The facts which support the parasitic theory of cancer may be summarized in the following way : In its initial stages the disease is purely local, then gradually it spreads to the adjacent tissues, and at the same time infects the lymph-glands which receive the lymphatics from the affected area, and general infection of the body (dissemination) follows. 230 EPITHELIAL TUMOURS Some writers refer to the toxic effects (cacliexia) exhibited by individuals with well-established cancer as evidence in favour of its parasitic origin; but Cooper believes that this toxic effect has been exaggerated, and points out that it is absent even in extensive cancer where no ulceration or external contamination is present. When micro-organisms gain access they find a malignant growth a favourable nidus for their development, and septic intoxication more or less rapidly ensues. This matter was discussed in the preced- ing chapter in relation to the manner in which death so often occurs in the cancerous by what are called terminal infections {see p. 268). In many instances cancer seems to have a period of quiescence, and then to enter on a period of recrudescence, exactly like a chronic infectious disease such as syphilis. The primary focus in this disease disappears after a time and leaves but little trace of its existence, so occasionally in carcinoma the primary focus may atrophy and become inconspicuous. The infectiveness and vitality of the cancer-cell have been already discussed, and form a strong argument for those who are seeking for a parasite ; but to my mind the most valuable evidence is supplied by the distribution of the initial lesions of cancer. When cancer arises on those parts of the body easily accessible to observation, such as the lips and tongue, it is always preceded by a wound, chronic inflammation, and especi- ally chronic syphilitic lesions. It is also recognized that the disease occurs most frequently in situations where there is access of air, and on free surfaces, as in the case of the intes- tinal tract ; and it is clearly established by a careful study of death-returns that in more than half the cases in which death is attributed to cancer the primary seat of the disease is in the digestive organs. The distribution of cancer in that part of the alimentary canal which occupies the belly is somewhat remarkable. For example, the stomach is not only the com- monest primary seat of cancer when compared with other digestive organs, but it stands third in order of frequency among all organs, the breast (mamma) being first and the uterus second in order of liability. The small intestine (duo- denum, jejunum, and ileum) is very rarely attacked- by cancer. CAUSE OF GANGER 281 but in the rest of the canal (colon and rectum) it is a very frequent disease. These facts give colour to the hypothesis that the cause of cancer is a micro-parasite conveyed by uncooked food or water. When men and women are impatient at our ignorance in regard to the cause of cancer, it is well to remind them that the part played by the spermatozoon in fertilizing the ovum has only been known about sixty years. Need we complain that it has been so difficult to find the cause of cancer ? It is most probably a micro-parasite which stimulates the normal epithelial cells of adult individuals to multiply and produce cancer in the same way that the male gamete or spermato- zoon initiates reproductive changes in the female gamete or ovum. The feature which distinguishes carcinoma from all in- fective diseases is its property of causing secondary deposits which reproduce the structural details of the organ primarily affected. This remarkable vitality of epithelium is, of course, exhibited in its highest form by the ovum, which is an epi- thelial cell, and one which, under certain conditions, exhibits malignancy in a very extraordinary form (Chap. XLix.). 3. The biologic theory and the cytologic transforma- tions observed in malignant tumours. — Among the most important observations which have been recorded in relation to cells of malignant tumours, attention must be given to those made by Farmer, Moore, and Walker in relation to nuclear division. It is known that, in the production of sexual (gametogenic) cells in plants and animals, the forms of nuclear division differ materially from those exhibited by cells which compose the tissues of the body (somatic cells). The above-mentioned investigators have been able to trace in detail a number of definite and serial changes in the cells of invading and pro- liferating malignant tissues which are remarkably similar to those obtained during the maturation of the elements contained within the sexual reproductive glands, and this resemblance extends to minute points of detail. These observations show that the various types of malignant growths present certain features in their cy to- logical transformations common to all, and that these features 282 EPITHELIAL TUM0UB8 are similar to those to be observed in the process of differentia- tion of reproductive cells from the preceding somatic tissue. The evidence, the investigators believe, justifies them in correlating the appearance of these " gametoid " neoplasms with the result of a stimulus which has changed the normal somatic course of cell-development into that characteristic of reproductive (not embryonic) tissue. These peculiar nuclear changes have not been observed in innocent tumours. The same investigators have succeeded in showing that the remarkable vesicular structures found in cancer-cells, known as " bird's-eye inclusions " or Plimmer's bodies, occur normally in cells during the production of sexual elements in vertebrates. These bird's-eye inclusions consist of a well- defined wall enclosing a clear fluid in which are suspended one or more darkly-staining granules. In size they may be very minute, or may equal the nucleus. One, or as many as twenty, may occur in the same cell ; they commonly lie adjacent to the nucleus, which they frequently press, giving it a crescentic appearance. These bodies are very conspicuous, and were long regarded as peculiar to malignant growths; they acquired some notoriety on account of their resemblance to Plasmodiophora hrassicce, discovered by Woronin in 1876 as the cause of a disease of the edible Crucifera?, especially cabbages ; this relationship, however, has not passed beyond the bounds of speculation. Now that Farmer and his co-workers have shown that the archoplasTnio vesicles, as they have been called, appear during spermatogenesis in all vertebrates, and are to all appearance structurally identical with and arise in a manner similar to the " bird's-eye inclusions" in the cells of cancer (Plimmer's bodies), it rather weakens beUef in their specificity for malignant growths. The peculiar nuclear changes observed in the cells of malignant growths do not affect all the cell-elements equally ; those which show the changes in the highest degree are " situated in a zone behind the growing edge of the advancing neoplasm." In the slow-growing tumours which produce a considerable amount of normal somatic tissue (fibrous tissue), cells showing the phases here referred to are far more difficult to find than CAUSE OF GANGER 283 in the rapidly growing tumours. In such growths, cells show- ing the figures of ordinary somatic division are numerous in comparison with those showing heterotype figures. This would seem to indicate that the cells which are destined to form fibrous tissue never divide heterotypically. These observers look upon this remarkable transformation as representing the immediate cause of development of the malignant growth, but the remote cause, the specific irritant, has yet to be found. Nevertheless, the interesting changes which they have detected constitute a valuable and inter- esting item in our knowledge of the cytology of malignant tumours. Bonney has shown that a gametoid type of mitosis occurs in the cells of intracystic papilloma of the ovary and in the cells of the gonorrhoeal wart. In describing the histologic features of primary and secondar}^ cancerous tumours, it was pointed out that the epithelium resembled that of the part in which the cancer arose primarily, and that in the case of a carcinoma arising in a glandular organ the cells not only resembled the cells of the gland, but the grouping of the cells, especially in the secondary deposits, was a mimicry, so to speak, of the gland itself. This peculiarity of carcinoma has attracted the close attention of all investigators who have made the structure of cancers a special subject of study, and this aspect of the matter has seemed to become more thoroughly established with each improvement and refinement in histologic methods, until it seemed to be a matter which did not admit of dispute. The subject has been carefully investigated by Cooper, who points out in regard to it that no one has witnessed on the stage of a microscope the actual conversion of a normal into a malignant cell, and reminds us that cells of an embryonic type, and possessing considerable powers of reproduction, are normally present in our tissues throughout life, and play an important part in what may be called tissue maintenance; and he ventures on the suggestion that cancer-cells are formed from the histogenic cells of the body, and are therefore most probably of a primary embryonic origin, but that they have departed morphologically and physiologically from the 284 EPITHELIAL TUMOURS normal type of the histogenic cell. The cancer-cell resembles its embryonic prototype from the fully formed, functionally active tissue-cell of the adult in the following particulars : — 1. Its generalized shape, which, although variable and irregular, inclines on the whole to be spherical ; often, however, the natural shape is altered by pressure. 2. Its comparatively large nucleus, which often indicates evidence of division. 3. Its more or less homogeneous protoplasm and the large proportion of glycogen. The cancer-cell differs from the normal prototype in several points, such as its simple method of cell-division ; powers of movement or migration ; ability to engulf albuminous particles, and its proneness to undergo degeneration. These observations support the view that cancer-cells are intrinsic to the body, and that they are derived from the pre-existing and presumably normal cells of the body. The careful histological study of malignant tumours reveals in a decided way that in whatever kind of tissue a sarcoma arises, its malignancy may be fairly gauged according to the degree in which it departs from the normal towards the round-celled type of tissue ; in the same way the greater the deviation of the epithelial cells of a cancer towards the spher- oidal cell, and the more it caricatures in the arrangement of the cells the structure of the gland in which it arises, the more dangerous is it likely to be to the life of the individual in whom it occurs. Perversions in type of this kind used to be expressed by the term ^metaplasia ; but there is a tendency to restrict this name to express the mutation of epithelium from a columnar cell to the flattened or squamous kind. The deviation of the tumour- tissues from the normal type towards the round cell in the case of connective - tissue tumours, and to the spheroidal cell in the case of epithelial tumours (carcinoma), is now conveniently expressed by the term ana.pUtsia., and it is possible to express this structural alteration in the form of a law : The degree of ctnaplasia exhibited hy a turnour rejjresents the d.egree of its malignancy. This is a scholastic form for expressing a fact long recognized, that the more a tumour diverges from the type of its matrix the greater the malignamcy. GAU8E OF CANCER 285 For many years after Yirchow taught that every tissue in a tumour had a physiological prototype, it seemed difficult to find a satisfactory example of the erosive power of the cancer- cells ; but the researches into the remarkable tumour known as chorion-epithelioma have taught that the trophoblast of the developing embryo resembles in this respect a mahgnant tumour, except that in health it affects a limited area of the maternal tissue; but when abnormal and excessive it exhibits malignancy in all its forms, recurrence after re- moval, wide dissemination and invasiveness. The strongest argument against the parasitic theory is the failure to cultivate the cancer-cell outside the body, and in this connexion reference may be made to the important observations and experiments of Jensen on tame mice. It appears that mice are liable to tumours which run a malig- nant course. Jensen has been able to transplant portions of the tumour into other mice with success through nineteen generations. The original tumour occurred sporadically in a white mouse, and although the transplantations were successful with various kinds of mice except those known as blue mice, the experiments succeeded best with white mice. Jensen's experiments have been repeated in London by Dr. Bashford, and similar results have been obtained. In order to emphasize the difficulty of what for conveni- ence may be termed the cancer question, it is necessary to mention that competent pathological and bacteriological investigators, who have conducted the most painstaking and laborious researches with the hope of discovering the cause of carcinoma and sarcoma, are divided into two camps, namely, those who strongly believe that it is due to a micro-parasite, either a bacterium or some lowly animal form such as a protozoon ; and those who think the disease is due to some altered conditions of the cells independent of parasites. The position for the non-expert in this matter is illustrated by the following lines from " Empedocles on Etna " : — " The gods laugh in their sleeve To watch man doubt and fear, Who knows not what to believe, Since he sees nothing clear, And dares stamp nothing false where he finds nothing sure.' 286 - EPITHELIAL TUMOURS AVhilst investigators are hunting for tlie cause of ma- lignant tumours, practical surgeons have to deal with the concrete disease. Bonney, V., " On Gametoid Types of Mitosis in the so-called Gonorrhoeal Wart."— .4r?#. Bart.'s Hosp. Repts., 1881, xvii. 205. Paul, F. T., " Cases of Adenoma and Primary Carcinoma of the Liver." — Trans. Path. Soc, 1885, xxxvi. 238. Slade, G. R., " Gall-Stones and CanceT."— Lancet, 1905, i. 1052. Terrier et Auvray, " Les Tumeurs du Foie au point de vue Chirurgical : litude sur la Eesection du Foie."— iZev. de Chir., 1898, xviii. 403, 706, 831. BEFEBENGE8 359 Thompson, J. E., "The Surgical Treatment of Neoplasms of the Liver." — Ann. of Surg., 1899, xxs. 284. Weber, F. P., and Michels, E., " A Case of Chronic Jaundice and Great En- largement of the Liver due to Primary Carcinoma of the Extra-Hepatic Bile-Ducts commencing at the Junction of the Hepatic Ducts." — Med. Chir. Trans., 1905, Ixxxviii. 247. White, W. Hale, " On Primary Malignant Disease of the Liver." — Guys Hosp. Repts., 1894, xlvii. 59. White, W. Hale, " Carcinoma of the Pancreas." — Clin. Joiorn., 1900, xvi. 97. CHAPTER XXXV CARCINOMA OF THE URINARY AND EXTERNAL GENITAL ORGANS THE URINARY ORGANS Evert part of the urinary system is liable to cancer — kidney, ureter, bladder, prostate, and urethra. It is common in the Fig. 172. — Cancerous kidney in section. From a man 54 years of age. bladder; next in order of frequency come the complex 360 GANGER OF THE KIDNEY 361 glandular organs — the kidney and prostate. It is rarest in the conduits — the ureter and the urethra. We shall find it convenient to consider each part in ana- tomical sequence, beginning with the kidney. Cancer of the kidney. — Carcinoma of this organ starts in the epithelium of the uriniferous tubules, and gradually transforms the renal tissue without violently distorting the shape of the gland (Fig. 172). The cancerous tissue creeps into the pelvis of the kidney and invades the ureter, ex- Fig. 173.— Microscopic characters of renal cancer. tending sometimes the whole length of the duct, and the out- runner has been observed to enter the bladder. This relation of the carcinoma to the ureter explains the frequency of hgematuria as a concomitant of this disease. The minute characters of renal carcinoma are very strik- ing, and consist of tubules lined with regularly arranged columnar epithelium (Fig. 173), and the general arrangement of these tubules in microscopic sections presents " a rough but striking resemblance to the tubular structure of the kidney " (Sharkey). Our knowledge of the oreneral characters of renal car- 362 EPITHELIAL TUMOURS cinoma is very limited, because it is only during the last ten years that any serious efforts have been made to separate the epithelial malignant tumours (carcinoma) of the kidney from the connective- tissue (sarcomatous) tumours, which are by far the most common form of malignant tumours that attack this gland. Renal carcinoma is uncommon before middle life and in- creases in frequency after the fiftieth year, and is usually Fig. 174. — Horseshoe kidney ; one half of the organ is the seat of carcinoma. From a woman 60 years of age. {Mmeion, Eoyal College of Surgeons.) limited to one kidney. The unilateral character comes out strongly when the disease attacks a horseshoe kidney, for even under these conditions it remains restricted to one halt of the compound gland (Fig. 174). Cancer of the kidney is a very deadly disease, and a careful study of the records relating to cases in which the his- tology of the tumour was carefully determined shows that many of the patients from whom the kidney is removed die from the direct effects of the operation. This is partly due to the exhausting effects of the cancer and hsematuria GANGER OF THE UBETEE AND BLADDER 363 upon individuals advanced in life. Of the patients who re- cover from the operation, it is exceptional for life to be prolonged more than a year. Cancer of the ureter. — The terminal orifices of ducts are not uncommon situations for primary cancer, e.g. the duodenal end of the bile-duct, the urethral orifice in both sexes, and the vesical as well as the dilated or pelvic portion of the ureters. It is, however, rare for cancer to arise in any part of the ureter between the renal pelvis and the bladder. Voelcker has recorded a case in which a primary carci- noma arose in the right ureter at the spot where it crosses the brim of the pelvis. The patient, a man 68 years of age, came under observation on account of hsematuria. At the post-mortem examination a tumour as big as a cherry, which on microscopic examination furnished the characters of carcinoma, was found in the ureter. The lymph-glands on the corresponding side of the aorta were infected. There was a large secondary mass of cancer in the liver, and there were nodules in the right lung, which agreed in their microscopic characters with the tumour in the ureter. Cancer of the bladder.— In this viscus cancer is of the squamous-celled variety, and arises in the mucous membrane. From what is known of the habits of this disease elsewhere, it would, be anticipated that in a certain proportion of cases it would begin at the orifices of the ureters. This is actually the case ; but it must not be assumed, when the ureteral orifices are found involved in the late stages, that the disease originated at these orifices. Cancer of the bladder seems to be more common in women than in men. The signs of its presence are hi3ematuria, painful micturition, and cystitis. Such signs are, of course, equivocal, and its presence is demonstrated by means of the cysto- cope. It is very unusual before the age of 40. Death results from renal complications, exhaustion from repeated bleeding, bodily suffering, and frequent micturition. Carcinoma has been observed on an extroverted bladder ; the patient was a man aged 60, who " had always earned his living by cracking stones" (NeAvland). Treatment. — Operations on the bladder are of two kinds : (1) those which are performed to relieve the patient of the 364 EPITHELIAL TUMOURS frequency of micturition and the attendant pain, and (2) those which are directed to the extirpation of the cancer. Operation of the first kind consists of cystotomy, either through the perineum or above the pubes. I have found the best consequences follow a suprapubic opening. The urine flows away as soon as it enters the bladder, and the patient quickly learns to manage the necessary tube and receptacle, and is not obliged to remain in bed. The more radical treatment consists either in removal of the tumour with the implicated segment of the bladder, or in complete extirpation of the vise us. In the case of women the ureters have been diverted into the vagina, and in the case of men into the rectum. It is a fact of some value that the rectum will accommodate a fairly large quantity of urine under such conditions. The results of complete removal of the bladder are not encouraging. Partial resection of the bladder is attended with better consequences, especially when the tumour is situated on or near its summit. Operative treat- ment of bladder-tumours is necessarily restricted, because in the majority of cases the tumour arises in the vicinity of the ureteral orifices. Cancer of the urethra. — ^This is an extremely rare situa- tion for cancer. Nevertheless, there are some carefully recorded cases. The disease is of the squamous-celled variety, and usually arises in that part of the urethra which is in relation with the bulb. The patients were between the ages of 50 and 73. The trouble in each instance attracted atten- tion as a hard mass in the perineum which interfered with micturition, and attempts to pass a catheter provoked great pain and induced free bleeding. The obstruction increased until the urethra became impermeable and fistulse formed in the perineum. In most of the cases perineal section was performed, and the cut surface of the tumour had a greyish- white appearance and was extremely brittle. Cancer of the urethra occurs, though rarely, in women : it may be of the columnar-celled or the squamous-celled type. The first variety ma}^ arise in the urethral recesses known as Skene's tubes. The free removal of the urethra in women for carcinoma usually entails incontinence of urine. (See Boyd.) SWEEP'S GANGER ' 365 Cancer of the prostate. — The prostate is occasionally affected by cancer, especially in old men. As the disease advances it extends beyond the prostate and infiltrates the tissues around the base of the bladder. The pelvic lymph- glands become infected, and dissemination is common. It would appear that secondary deposits in bone are a very con- stant feature of prostatic cancer, and it has been particularly studied by von Recklinghausen. The radical treatment of cancer of the prostate is beyond surgical art. The enlargement of the prostate, which is so common after middle life, and is often termed prostatic adenoma, is the result of a slow, chronic inflammatory change. This subject has been very thoroughly handled by Ciechanowski (1903). THE EXTERNAL GENITAL ORGANS The greater part of the external genital organs are directly continuous with and derived from the skin ; they are liable to squamous-celled cancer. Cancer of the scrotum (sweep's cancer). — This appears on the scrotum in the form of a wart or warts ; they are often spoken of as soot-warts, for they not only occur on the scrotum of the chimney-sweep, but are met with in men who are brought much in contact with soot. In many cases the scrotal wart is harmless, but in a certain proportion of cases it grows slowly, or if multiple, one becomes more prominent than its fellows and ulcerates. The ulceration, at first limited to the wart, extends to the surrounding skin and forms a cancerous ulcer, which will extensively involve the scrotum, spread thence to the skin around the anus and pubes, and even to the thigh. In some cases the ulceration, instead of spreading widely, involves the tissues deeply, so that the tunica vaginalis is exposed and sometimes implicated in the disease ; but this is rare. The inguinal glands become infected and attain a large size^ then slowly involve the skin, break down and ulcerate. This process often leads to the formation of deep excavations in the groin, and it not infrequently happens that the femoral or the external iliac artery, or both, will be seen exposed and 366 EPITHELIAL TUMOUBS pulsating on the floor of one of these deep pits. It is not uncommon in such cases for the ulceration to open up one of these large vessels, and violent, fatal hsemorrhage is the result. It has been stated by several writers that in chimney- sweeps cancer may begin in the inguinal glands. There can be little doubt that such views arise in imperfect observation. In some of these cases the lesion on the scrotum assumes the form of a small hemispherical pimple no larger than a split pea — so small, indeed, that I have known it escape very vigilant eyes — and yet such a small lesion will cause the inguinal lymph-glands to grow into a mass fully as big as two fists. Two cases of this kind have come under my own notice. A very remarkable feature connected with cancer in English chimney-sweejjs is, that they are not more prone to it in other parts of their bodies than those persons who follow other occupations ; yet the scrotum, which in other indi- viduals is the part least disposed to cancer, is in sweeps so very hable to become the seat of this disease. No answer to this problem is at present forthcoming ; neither has anyone succeeded in assigning a reason why it is so very much more frequent m English chimney-sweeps than in sweeps of other nations. There is good reason to believe that tar and paraffin are liable to produce an affection of the scrotum similar to sweep's cancer. Such cases are, however, very rare. The literature has been summarized by Butlin. Treatment. — This consists in the free removal of the disease whenever it is practicable ; the very best results follow the excision of a soot-wart in its earliest stages. When the disease is permitted to extend deeply into the tissues of the scrotum, so that it is necessary to excise one or both testicles with the scrotum, and perhaps a portion of the neighbouring skin, it is not probable that lasting benefit will follow the operation. In cases where soot-warts have been early and thoroughly removed there is good ground for the belief that a cure is sometimes brouo-ht about. Cancer of the testis, — This subject is discussed in Chap. Liii. GANGER OF TEE PENIS 367 Cancer of the penis. — This disease may attack the prepuce or the epithelial investment of the glans. Carcinoma arising in the epithelium of the urethra is considered on p. 364. The disease is excessively rare before the age of 30 years, and appears to be most common between the ages of 50 and 70. Phimosis, congenital or acquired, appears to be a condition that favours cancer of the penis. It is certainly true that phimosis, by leading to the retention of smegma, is indirectly a cause of penile warts not only in men but other mammals, especially horses and bulls. Mention has already been made of the fact that penile warts are particularly prone to be trans- formed into wart-horns, and cases have been recorded in which men have had a wart-horn on the penis for several years, and at length its base has become the starting-jDoint of cancer. It must be remembered that cancer may begin as an ulcer on the penis, but the warty variety is by far the more frequent. When the disease begins as an ulcer, it is very liable to be mistaken for some manifestation of primary or tertiary syphilis. On the other hand, very great care must be taken not to mistake a breaking-down gumma of the glans penis for cancer. Cancer, in whatever form it begins, gradually involves and as surely destroys the penis, implicates the scrotum, and infects the inguinal lymph-glands on each side ; in many cases the lumbar glands also become infected. Secondary deposits seem to be rare. The duration of life in this disease is very uncertain. As a rule, its course is short — six months to a year ; but in many cases it is much longer. When the urethra is involved this passage becomes narrowed, and not infrequently urinary fistula3 add to the patient's misery. Cancer of Cowper's glands. — These structures are liable to inflame and become cystic, and there is also reason to believe that the gland may become cancerous. The most recent contribution to the subject is by Witsenhausen. Treatment.- — Cancer of the penis is treated by partial or complete removal of this organ, according to the extent of the disease. Partial removal of the penis is a simple proceeding, and entails but little risk so long as the cut end of the urethra is stitched to the skin. When the disease is so 368 EPITHELIAL TIJMOUBS extensive as to demand complete removal of the penis, the operation which gives best results consists in excising not only the corpus spongiosum and corpora cavernosa, but the penile crura as well, by detaching them from the pubic arch. The urethra is brought out and attached to the incision in the perineum. In all cases where it is justifiable to amputate or extirpate the penis for cancer the infected inguinal lymph- glands should be thoroughly removed. The published results of this complete operation are very good, and my experience of it has been in ever}^ way satisfactory. The ultimate results of amputation of the penis are more favourable after partial than after complete removal of the organ, simply because the disease is not so advanced when partial amputation is sufficient. Cancer of the vulva and vagina. — The variety of cancer which attacks the external genital organs of the female, with the exception of Bartholin's glands, is squamous-celled. Collectively, cancer of these parts is not uncommon, but when each part is individually considered, then it is compara- tively rare. The disease is more frequent in the labia than in all other parts of the genital passage taken together. The labia majora and minora. — Carcinoma may begin on any part of the labia, but it generally attacks the opposed, or so-called mucous, surfaces. In many cases this is preceded by leukoplakia, identical in appearance and structure with lingual leukoplakia. Careful inquiries in London indicate that cancer of the vulva is as common as cancer of the lip in men. During the decade 1898-1908 fifty-eight women were admitted into the Chelsea Hospital for Women and the Middlesex Hospital with carcinoma of the vulva, and in all the patients the inner surfaces of the labia majora presented the condition known as leukoplakia. When the social histories of these women are analysed they are instructive, because among the fifty-eight patients there were sixteen widows, thirty- four married women, and eight spinsters. These observations indicate that trauma connected with coition and childbirth are probably factors in producing the changes which render the epithelial tissues of the vulva liable to cancer. The relation of leukoplakic vulvitis and kraurosis of the GANGER OF TEE VULVA 369 vulva to cancer lias been carefully investigated by Comyns Berkeley and Victor Bonney. In this valuable monograph they point out that kraurosis is not a forerunner of cancer. It is a significant feature in relation to vulvar cancer that trauma connected with the sexual act and its results plays the same part in connexion with the labia that the habit of smoking short, dirty clay pipes exercises on the mucous membrane of the lips. The disease runs a course very similar to squamous-celled cancer of the scrotum. When recognized in the early stages, prompt and free excision and removal of the infected inguinal lymph-glands is followed by much the same suc- cess which attends operations upon cancer of the lip. In operating for cancer of the vulva the method which has given me the best results, immediate and remote, consists in freely excising the primary disease with the scalpel. Healing usually takes place in fourteen days. The lymph-glands, large and small, are then removed from both inguinal regions. Dividing the operation in this way avoids the risk of sepsis and diminishes shock and hsemorrhage, for in many instances operations on the vulva are attended with free bleeding. The clitoris.— Cancer of this organ is a rare disease; the majority of the patients are over 50 years of age. One example has come under my notice, and in this the disease began at the free extremity of the clitoris, in a woman 45 years of age. The treatment consists in free removal of the clitoris and its crura, and removal of infected inguinal lymph-glands. If the operation is carried out before the disease has extended to the nymphse, labia, or mons, the outlook for the patient is favourable. Bjorkquist has collected sixty-seven cases from the litera- ture. He considers the prognosis grave : in twenty patients death occurred in sixteen months. The vagina. — Carcinoma may attack any part of the mucous membrane hning this canal, but it is much more prone to begin at the vulvo -vaginal junction. In the majority of cases which have come under my observation the cancer has been in the immediate vicinity of the urethral oriiice. In every instance the patients were past middle life, and one was Y S70 EPI'TliELlAtj TtJMOURS 73 years old. The inguinal lymph-glands are early infected. The cancer quickly implicates the vesico-vaginal septum and leads to fistula, and when it attacks the posterior wall it causes a recto-vaginal fistula. In one case the urethral orifice became blocked with cancerous granulation, and retention of urine was a very distressing symptom. In the early stages cancer of the vagina produces so little inconvenience that the patients do not seek advice until the disease is far advanced. Surgery can do little in cancer of the vagina, for even in the very early stages free removal may anticipate some of the evils of the disease by establishing a vesical or a rectal fistula. Cancer of the vagina is rare ; for example, in the quin- quennium 1904-8 three patients with cancer of the vagina died in the Middlesex Hospital. During the same period 241 women with cancer of the neck of the uterus were admitted to the general wards and to the Cancer Asylum of this hospital. {Eighth Report of the Cancer Research Laboratories.) Bartholin's glands. — It is well known that these glands are very liable to become cystic ; they are prone, too, to septic infeation. They are also occasionally the source of cancer. Schweizer has reported a case and collected the literature. Beck, Marcus, "A Case of Primary Squamous Carcinoma of the Bulbous Portion of the Urethra." — Internat. Clinics, 1893, ii. 256. Berkeley, Comyns, and Bonney, V., "Leucoplakic Vulvitis and its Eelation to Kraurosis Vulvae and Carcinoma Vulvse." — Brit. Med. Journ., 1910, ii. 1739 Bjorkquist, " Festschrift gewidmet Otto Engstrom," Berlin, 1903. Boyd, Mrs., " Tvfo Cases of Primary Carcinoma of the Female Urethra." — Journ. of Oistet. and Gyn., 1906, ix. 40. Butlin, H. T., " Cancer of the Scrotum in Chimney-Sweei^s." — Brit. Med. Journ., 1892, i. 1341. Ciechanowski, S., " Anatomical Researches on the so-called Prostatic Hyper- trophy," 1903. Dittrick, H., " Epithelioma of the Vulva, with references to the Literature." — Amer. Journ. of the Med. ScL, 1905, cxxx. 277. Griflaths, J., " Epithelioma of the Male Urethra." — Travis. Path. Soc, 1889, xl. 177. BEFEBENGES 371 Newland, H. S., " Extroversion of the Bladder." — Brit. Med. Journ., 1906, i. 9G6. von Recklinghausen, F., " Die fibrose oder deformirende Ostitis, die Osteo- malacia, und die Osteoplastische Carcinose in ihren gegenseitigen Beziehungen." — Festschrift Rudolf Virchow zu seinern 71 Geburtstage gewidmet, Berlin, 1891. Schweizer, Fritz, " CarcinomderBartholiniscbenDriise." — Arcli.f. Gyn., 1S93, xliv. 322, Tuffier et Dujarier, "Extirpation Totale de la Vessie." — Rev. do Chir., 1898, xviii. 279. Voelcker, A. F., " Primary Carcinoma of the Ureter." — Trans. Path. Soc, 1895, xlvi. 133. Witsenhausen, 0., "Das Primare Carcinom der Urethra." — Beit. z. Idin. Chir., 1891, vii. 571. CHAPTER XXXVI EPITHELIAL TUMOURS OF THE UTERUS The endometrium of the cervical canal and body of the uterus is covered with columnar epithelium ; it is continued through the Fallopian tubes to end at their coelomic ostia, Fig. 175. — Microscopic characters of the pink tissue at the neck of the uterus, commonly called an " erosion." {After Bonney.) where there is an abrupt transition to the pavement-like epithelium (endothelium) of the peritoneum. The epithelium of the cervical endometrium undergoes transformation at the external mouth (or os) of the uterus into the stratified or squamous-celled type which lines the vagina. The columnar cells within the uterus and Fallopian tubes are ciliated. Changes occur throughout any portion of this epithelial tract, but vulnerability of the epithelium varies greatly in the different regions. 372 ADENOMA OF THE UTERUS 373 It will be convenient to study the epithelial changes which are non-malignant before considering those of a cancerous kind. Adenomas. — The endometrium of the cervical canal is furnished with numerous racemose glands. Adenomas, which are structurally repetitions of these glands, are very common at the neck of the uterus; they may be sessile or pedunculated. A sessile adenoma appears as a soft velvety areola around the os; it is in colour like a ripe strawberry, and thickly dotted with minute spots of a brighter pink. This Fig. 176. — Tubular glands of the corporeal endometrium in transverse section. pink tissue is composed of glandular acini lined with large, regular, columnar epithelium (Fig. 175). The glandular tissue often extends beyond the margins of the os and invades the vaginal portion of the cervix. Sometimes it is so abundant that the apex of the cervix, instead of being a cone, assumes rather the shape of the under surface of a mushroom. The glandular mass is not confined to the margins of the os, but extends for a variable distance up the canal. When adenoma affects a lacerated cervix the whole of the exposed portion of the canal is involved. The surface of a sessile adenoma is 374 EPITHELIAL TUMOURS covered witli tenacious mucus secreted by the abnormal glands. Pedunculated adenomas are rarely large : they may grow from any part of the cervical canal, but are most frequently found springing from the lower 2 cm. of the canal. As a rule they occur singly, but two or more may be present. They Fig. 177. — Uterus with villous disease of the endometrium. Removed from a multi- para aged 83. The patient was alive and in good health three years later. are soft and velvety to the touch, and dotted with minute pores. They consist of an axis of fibrous issue, covered with mucous membrane continuous with that lining the cervical canal. When these pedunculated adenomas (polypi) remain within the canal, the epithelium covering them and the glands they contain are of the same character as those of the cervical mucous membrane. When the tumours increase in size and project into the vagina, the epithelium covering the protruding portions becomes stratified and the glands disappear. ABENO-MYOMA OF THE UTERUS 375 Adenomas growing from the corporeal endometrium are pedunculated, and so soft that they are often termed mucous polypi. Microscopically these tumours consist of cystic spaces lined with columnar epithelium, the cavities being filled with mucus. Adenomas of the corporeal endometrium differ from those of the cervix in that the cystic spaces are larger and more numerous (Fig. 176). Fig. 178. — rrterus in sagittal section showing diffuse adeno- myoma, from a spinster 32 years of age. The gland s^Daces were cystic and filled with gelatinous material. A rare epithelial change in the endometrium is known as papilloma of the endometrium. In this condition the uterine cavity is filled with villi (Fig. 177). Each of these has a delicate axis of vascular connective tissue covered with a single layer of columnar epithelium. The clinical signs are similar to those caused by cancer of the corporeal endometrium. Adeno-myoma. — This term is applied to a pathologic con- dition of the uterus the leading features of which have been admirably summarized by Cullen in the following terms: " It is diffuse in character, situated in the middle layer of 376 EPITHELIAL TUMOURS the uterine wall, and is dependent on the uterine mucosa for its glandular elements." Although several observers, in- cluding von Recklinghausen, have recorded isolated examples of this disease, Cullen seems to have been the first to draw attention to its clinical importance (1897). In well-marked cases adeno-myoma presents clinical features which cause it to resemble the common varieties of submucous fibroids. The ages of the patients vary from 20 to 50 years ; the uterus is usually enlarged, but in Fig. 179. — Microscopic features of diffuse adeno-myoma of the uterus. X 60. {Frraik E. Taylor.) exceptional instances adeno-myoma occurs in small atrophic uteri. Adeno-myomatous changes are often associated with fibroids. Small bodies under the serous coat of these uteri often resemble stalked subserous fibroids ; they are really " buds " of adeno-myomatous tissue. When the uterus is removed and divided longitudinally, the walls are seen to be greatly thickened, measuring in some specimens 5 cm. (2 in.) in thickness ; this increase is due to the formation of new tissue between the outer wall of the uterus (the subserous stratum) and the superficial layer of the endometrium. There is no attempt at encapsulation, and the term "diffuse" is thoroughly justified. (Fig. 178.) ADENO-MYOMA OF TEE UTERUS 377 The cut surface of the adventitious tissue differs from that presented by the common hard fibroid in another particular, for, instead of forming the well-known vortex arrangement, the muscular tissue is disposed in an irregular manner, and on the freshly cut surface it produces a pattern not unlike that of the fabric known as " watered silk." The new tissue consists mainly of bundles of plain muscle- fibre, which, instead of being arranged in vortices, as is so Fig. 180. — Uterus in section showing diffuse adeno-myomatous disease. The poly- poid process contains glandular elements. From a spinster aged 43 years. common in the ordinary hard fibroid, are disposed in an irregular manner, and the spaces between the bundles are filled with the peculiar stroma of the uterine mucosa, containing gland-tubules lined with columnar epithelium of the same type as the normal tubular glands of the endo- metrium. The glandular elements appear to be uniformly distributed throughout the adventitious tissue, and can be detected up to the limits of the thin muscular stratum underlying the peritoneal coat of the uterus. The amount of glandular tissue varies greatly in different cases. (Fig. 180.) 378 EPITHELIAL TUMOURS An interesting featnre of the disease is the frequency with which uteri, the seat of this change, afford evidence of antecedent inflammation. The Fallopian tubes are frequently thickened and their ccelomic ostia occluded ; the uterus is in many cases firmly adherent to the bladder and intestines. It is quite possible that adeno-myoma of the uterus is due to a micro-organism, for several specimens have been de- scribed, in which uteri, the seat of this change, have also been Fig. 181. — Uterus laid open by a vertical incision. The endometrium on the anterior waU is occupied by an unencapsuled mass of tuberculous adenomatous tissue. From a spinster aged 46 years. The patient was in excellent health four years after the operation. tuberculous. The symptoms produced, by such complex changes are like those which accompany a degenerating sub- mucous fibroid. Specimens of this kind, have been described by Archambault and Pearce in New York, by Grlinbaum in Berlin, and by myself in London. Clinically, adeno-myoma of the uterus is liable to be mis- taken for submucous or interstitial fibroids. After operation it requires the use of a microscope for identification; even then many specimens have been mistaken for cancer of the corporeal endometrium. ADENO-MYOMA OF THE UTERUS 379 The leading clinical features may be summarized thus : Excessive menorrhagia and profound anaemia in women between the twentieth and fiftieth years, usually accompanied by an enlargement of the uterus like that which is caused by a submucous fibroid. Diffuse adeno-myoma of the uterus occurs in spinsters and in barren married women as well as in those who are fertile. In forty-nine cases examined by CuUen, " nine patients were spinsters and forty were married ; of these six were sterile, two had had miscarriages, and thirty-two had had children." Among the sixteen cases observed by Grlinbaum, six had borne children. Of the fifteen cases under my own care, seven of the patients were spinsters, and of the eight married women- four were mothers and one had been delivered of fourteen living children. Treatment. — When the patient's health is undermined from the prolonged and excessive bleeding which is associated with this disease, the uterus should be removed. Vaginal as well as abdominal hysterectomy gives excellent results, im mediate and remote. Archambault, J. L., and Pearce, R. M., " Taberculose d'un Adenomyome de rUterus." — JRev. de Gyn. et de Chir. Abdom., 1907, xi. 3. Bland-Sutton, J., "The Position of Abdominal Hysterectomy in London," 1910. Cameron, S. J. M., and Taylor, F. E., "On Adenomyoma of the Uterus." — Journ. of Obstet: and Gijn. of Brit. Emp., 1904, v. 248. Cullen, T. S., "Adenomyoma of the Uterus," Philadelphia, 1908. Griinbaum, D., "Adenomyoma Corporis Uteri mit Tuberculose. " — AreJi. f. Gyn., 1907, Ixxxi. 383. Griinbaum, D., "Clinical Features of Adenomyoma." — Milncli. med. Woch., 1908, p. 1156. Lockyer, C, "Three Cases of Adenomyoma Uteri." — Trans. Obstet. Soc, 1906 slviii. 84. Tate, W. W. H., " Two Cases of Diffuse Adenomyoma of the Uterus." — Trans. Obstet. Soc, xlvi. 141. CHAPTER XXXYII EPITHELIAL TUMOURS OF THE UTERUS (Continued) CARCINOMA OF THE NECK AND BODY OF THE UTERUS Carcinoma of the neck of the uterus. — This part of the uterus is liable to both squamous-celled and columnar- celled cancer, according to the situation in which it arises. When ^/■ft^^'^i^^— ^-r^^Si^^^^ Fig. 182. — Microscopic characters of the epithelium covering the vaginal aspect of the neck of the uterus. the disease begins on the vaginal aspect of the neck of the uterus, it is of the squamous-celled species ; if its origin is in the cervical endometrium, the cells will be columnar (Figs. 182 and 183). A large amount of energy has been devoted to the micro- scopic examination of cancer of the neck of the uterus, in the hope of determining the relative h-equency of the squamous- celled and of the columnar-celled variety. So far as my own efforts are concerned, they were directed with the object of deciding, if possible, which variety gave the best results to 380 GANGER OF THE UTERUS 381 operation ; but after a long and laborious investigation I came to the conclusion that it was hazardous to attempt a prediction simply on the cell-features of the cancer. Although the ultimate results of cancer arising in the cervical endometrium, or on the vaginal aspect of the cervix, are the same, it will be advisable to discuss their pathologic Fig. 183. — Microscopic characters of a gland from the cervical endometrium. features separately. In the majority of patients who come under observation, particularly in hospital practice, the disease has already destroyed, or eroded, the neck of the uterus to such an extent that it is impossible to determine whether it arose in the cervical canal or on the vaginal surface ; neverthe- less, patients do occasionally come under observation at a sufficiently early stage to enable an exact localization of the 382 EPITHELIAL TUMOURS primary focus of the disease to be made. It may appear as a circular ulcer "witli raised and everted edges, or it erodes the tissues deejDly at the outset ; exceptionally it forms luxuriant warty excrescences. The cancer infiltrates the cervix, extends to and implicates the vaginal wall, and involves the tissues of the mesometrium. Cancer also arises in the epithelium in any part of the cervical canal or its glands, but it aj)pears to be more prone to arise in the lower Fig. 184. — Microscopic characters of cancer of tlie cervix. than in the upper half of the canal. It begins either as a deeply eroding ulcer, or as a soft, fungating, vascular, cauli- flower-like outgrowth. Commonly the cancer, after infiltrating the adjacent tissues of the cervix, spreads into the meso- metrium and imj)licates the vaginal waU. It ulcerates early, and destroys the cervix and spreads into the body of the uterus, and in the late stages this organ may become hoUowed out by ulceration until nothing remains but a thin layer of muscle-tissue covered by peritoneum. When a uterus hoUowed out in this way has its cervical canal obstructed C ANGER OF THE UTERUS 383 by cancer, tlie uterine cavity becomes distended with pus. This condition is known as iiyometra. The pus sometimes escapes intermittently. The microscopic features of cancer arising in the cervical epithelium consist of round spaces filled with columnar epi- thelium. This depends on the fact that the invasion of the tissues is due to columns of epithelium, and in the micro- Inflltrated ovary. Uterine cavity. Wall of bladder. - Cancer. Cervical canal. Vagina. Fig. 185. — Cancerous uterus in sagittal section. scopic sections these cell-columns are represented cut at right angles (Fig. ]84). Cancer of the cervix leads to infection of the lumbar lymph-glands. Dissemination is also frequent, and secondary deposits form in the lung, liver, and occasionally in the bones, but not with the same frequency as in cancer of the breast. Cancer of the cervix leads to perforation of the anterior and posterior vaginal septa, so that urinary and fsecal fistulas are apt to complicate the late stages of the disease (Fig. 186). When the broad ligaments are extensively infiltrated the 384 EPITHELIAL TUMOURS ureters become involved ; this leads to dilatation of the renal pelves. Cystitis is a common complication of carcinoma of the cervix, and causes suppurative pyelitis and nephritis. A very large proportion of patients affected with cancer of the uterus exhibit marked ursemic symptoms in the later stages of their lives. Among other complications of cancer of the cervix, espe- Fig. 186. — Pelvis and its viscera in section. From a case of cancer of the uterine cervix which invaded the bladder. cially when it extends to the body of the uterus, must be mentioned pyosalpinx and hydrosalpinx. In these cases the dilated tubes are rarely thicker than the thumb, but they are a source of danger, inasmuch as perforation occasionally occurs and sets up infective peritonitis. Exceptionally the cancer perforates the body of the uterus. When this happens peritonitis may ensue and quickly cause death; in some GANGER OF THE UTERUS 385 instances tlie carcinomatous material becomes distributed over the peritoneum, and small knots form upon tbe serous surfaces of the intestine, liver, spleen, etc. This distribution of the cancer may lead to an effusion of blood-stained fluid into the belly, sometimes in considerable quantity; or to agglutination of coils of intestine, each cancerous nodule being the focus of a limited area of peritonitis. Occasionally A/i'^<^" Fig. 187. — Cancerous uterus ia coronal section. It was difficult to decide whether the cancer began in the upper part of the cervix or in the lower part of the body of the uterus. A process of the growth is creeping into the right Fallopian tube. actual perforation of the uterus is prevented by a piece of intestine becoming adherent to the uterus at the spot where the disease is approaching the surface : adhesion in this way rnay take place between the uterus and the small intestine. It is important to bear this in mind, because when a fgecal fistula complicates cancer of the uterus it is usually attributed to a communication with the rectum or sigmoid flexure, and these are the common situations; but in some cases the fistula is in the transverse colon, for when this section of z 386 EPITHELIAL TUMOURS tlie large bowel is omega shaped the lower segment of the loop often comes in contact with the fundus of the uterus. Cancer of the cervix uteri is very common between the ages of 40 and 50 ; many cases occur between 30 and 40. Before the age of 30 the disease is rare, but I have observed undoubted cases in women of 23, 25, and 26 years of age It belongs especially to the latter part of the child-bearing period of life ; it is almost exclusively confined to women who have been pi^egnaAit Critical inquiry shows that injury associated with coition and child-birth, but more particularly the latter, is a potent factor in producing the changes which render the epithelium in this situation liable to cancer, and it is disappointing to find that fecundity in- creases this liability. A remarkable record bearing on this matter has been published by Czerwenka. A woman 35 years of age had a double vagina and uterus bicornis bicollis. Coitus was practised in the left vagina. The left cervix was cancerous, the left uterus contained two fibroids, and the corresponding Fallopian tube contained pus and had its coelomic ostium occluded. The signs of cancer of the cervix are bleeding, offensive discharges, and sometimes pain. The first two signs are those which usually lead women to seek advice. In the early stages the margins of the os will be found everted, and a fungous mass protrudes from the canal, which bleeds on the slightest touch. In the late stages, when the neck of the uterus is destroyed and replaced by an ulcerat- ing cancerous mass, there is no difficulty in recognizing the nature of the lesion. Cancer of the uterus terminates in a variety of ways : — 1. The uterine artery may be opened by ulceration, and fatal hsemorrhage ensue. 2. Repeated bleeding due to smaller arteries being eroded will often lead to exhaustion and death. 3. Implication of the bladder and one or both ureters causes cystitis, septic pyelitis, and uraemia. Some observers fix the frequency of renal complications in this disease as high as 70 per cent. 4. Septic changes in the uterus extend to the Fallopiaii.- tube an4 cf\,use pyosalpinx. CANCER OF THE UTERUS 387 5. Peritonitis may be caused by rupture of a pus-containing Fallopian tube. 6. Intestinal obstruction may follow adhesion of a piece of small or large intestine to the uterus, or direct extension of the cancer into the rectum. 7. Hydroperitoneum and hydrothorax may arise from the presence of secondary nodules of cancer on the peritoneum or pleura. 8. The cervical canal sometimes becomes occluded, and the Ovary infiltrated with cancer. Fallopian tube. Eound ligament. Occluded ureter. Cancer-mass. Vesical orifice of ureter. Fig. 188. — Cancer of the neck of the uterus implicating the bladder and the ureter. cavity of the uterus becomes distended with pus (pyometra). The chief danger in this complication is due to the Fallopian tubes becoming secondarily distended with pus, which occa- sionally leaks into the peritoneum, with lethal results. Cancer of the cervix is sometimes complicated with other lesions of the genital organs, such as ovarian cysts and tumours, fibroids, etc. 388 EPITHELIAL TUMOURS Treatment. — The only treatment available for cancer of the neck of the uterus is early removal of the whole uterus. This is only practicable in a small percentage of patients, because the disease arises and spreads so insidiously that the cancer, in the majority of cases, has overrun adjacent parts, such as the vagina, bladder, rectum, and vesical segments of the ureter ; this precludes operative interference. In recent years methods have been introduced by Ries, Mackenrodt, Duhrssen, and Wertheim which enable the surgeon to remove not only the uterus and its neck, but the broad ligaments, Fallopian tubes, lymph-nodes and para-uterine connective tissue. These very extensive operations are attended with a high mortality. Palliative treatment. — In many cases where no operation is possible, much may be done to make the patients comfortable. Careful nursing keeps them clean, free from bed-sores and fetor ; a difficult matter when a woman has a faecal or a urinary fistula, or both. Pain may be alleviated by phen- acetin or the judicious use of morphia. Carcinoma of the cervix and pregnancy. — It may be stated without fear of contradiction that the most appalling com- plication of pregnancy is cancer of the cervix. It is some- what difficult to understand how a woman with cancer of the neck of the uterus can conceive, but it is quite certain that it happens, and that the complication is not uncommon. How- ever, cases in which cancer in this situation obstructs delivery are unusual, and this is due to two circumstances : 1. Cancer of the neck of the uterus predisj^oses to abortion. 2. When it has advanced to such a stage as to fill the vagina with an obstructive mass, the disease has such an effect upon the health of the mother that the life of the foetus is imperilled. The second condition is of importance, because in con- sidering the advisability of Csesarean section in these circum- stances it is well to be satisfied that the foetus is alive. However, in very exceptional cases it has been found necessary to resort to this operation in order to deliver a dead and putrid foetus. The careful study of the literature relating to this compli- cation shows clearly enough that when a pregnant woman GANGER OF THE UTERUS 389 with recent cancer of the uterus comes under observation in the early months, her best hope Hes in vaginal hysterectomy. In the later stages (fourth to the seventh month) very good consequences have followed amputation of the cervix, and this operation has been successfully performed without dis- turbing the pregnancy. In the latest stages the best conse- quences have followed the induction of labour and the immediate performance of vaginal hysterectomy — for, surpris- ing as it may seem, the uterus enlarged by the pregnancy can be safely extirpated through the vagina. These methods of treatment only apply to cases where the cancer is in such a condition as to afford reasonable hope of a prolongation of life. When the disease is in an inoperable stage and the foetus is dead, then after a little patient waiting abortion usually occurs. Where there is reliable evidence that the foetus is alive, the pregnancy should be allowed to go to term ; if the cancer affords an impassable barrier to the transit of the child, then Ctesarean section becomes a necessity. Cancer of the body of the uterus. — This is much less fre- quent than cancer of the neck of the uterus. It arises in the epithelium lining the uterine cavity. There is very little accurate knowledge regarding its early stages, and the writer has had only one opportunity of obtaining a cancerous uterus before the disease had extended to the muscular wall. The disease remains for a long time restricted to the body of the uterus, and may creep into the uterine sections of one or both Fallopian tubes ; it rarely invades the cervix, and then only in the late stages of the disease. It is apt to perforate the wall of the uterus and infect the peritoneum (Fig. 189). It is only during the last fifteen years that the importance of cancer of the body of the uterus has been clearly appreciated. This was due to the fact that there were no means available for the proper examination of the interior of the organ, and as a result the descriptions of diseases of the endometrium were disfigured or obscured by a crowd of terms such as senile endometritis, malignant endometritis, villous endometritis, and so on. When the plan of mechanically dilating the cervical canal was introduced, so that the endometrium could be examined and fragments obtained for the laboratory, then light began to shine, and we obtained some accurate data. 390 EPITHELIAL TUMOURS As in other organs, cancer of the body of the uterus consists of cell-cohimns, the cells beino- identical with the epithelial cells of the endometrium. The disease assumes two distinct forms . Thus it may appear as an eroding ulcer pene- trating the muscular wall of the uterus, and sometimes even perforating the serous coat (Fig. 189). In the common form it gives rise to luxuriant masses of soft, succulent, and vascular polypus-masses projecting into the cavit}^ of the uterus (Fig. 190) ; this is the variety which used to be termed villous endometritis. Fig. 189. — Cancerous uterus in sagittal section. A bud-like process of cancer has eroded the uterine wall and protrudes oa the peritoneal sm-face. The peri- toneum was dotted with thousands of secondary nodules. As the diagnosis of cancer of the body of the uterus is largely determined with the assistance of the microscope, it is essential for those who venture to give opinions on this point to be thoroughly familiar with the various abnormalities of the corporeal endometrium, and especially those which are known as glandular polypi. Although in writings and in clinical Avork we treat very definitely of cancer of the cervical endometrium and cancer of the corporeal endometrium, it is well to understand that GANG Ell OF THE UTERUS 391 cases come to hand in which, after the uterus has been removed, it is extremely difficult on examining the organ to state positively whether the disease arose in the body ot the organ or in the upper segment of the cervical canal (Fig. 187). Cancer of the corporeal endometrium is unusual before the forty-fifth year ; it is most frequent at or subsequent to the menopause. The majority of the cases occur between the fiftieth and seventieth years. A large 'proportion of the patients are nulliparoi. Fig. 190. — Uterus with "tubular" cancer, shown in coronal section ; the patient was 41 years of age, and mother of one chUd. The patient's attention is usually attracted by fitful haemorrhages after the menopause, followed by profuse and offensive discharoes which are often blood-stained. The uterus on examination may feel scarcely enlarged ; sometimes, however, it is much bigger than usual. In some instances cancer of the corporeal endometrium is associated with fibroids. Treatment. — Cancer of the body of the uterus entails com- plete removal of the organ, including its neck, by the abdomi- nal route. The ovaries, Fallopian tubes, and broad ligaments S92 BPtTHELIAL TUMOURS are removed with the uterus. If the lymph-nodes of the pelvis are enlarged and signs of dissemination are obvious on the peritoneum, omentum, or intestines it is useless to remove the uterus. Hysterectomy for cancer of the . body of the uterus is followed by better consequences, immediate and remote, than when this operation is performed for carcinoma of the cervix. Variations in malignancy. — Cancer varies widely in its malignancy in nearly all the situations in which it grows. As a rule, cancer in the neck of the uterus runs its course more rapidly than the same disease inside the uterus. This is due in a large measure to accidental circumstances, espe- cially to the facility Avith which cancerous tissue becomes septic. Cancers are not encapsuled, and grow, as a rule, on an epithelial surface exposed to micro-organisms. Cancers of the neck of the uterus become septic more quickly than those within the uterus. Observation also shows that cancer of the corporeal endometrium becomes septic more quickly in a multiparous than in a barren woman. The objective sign of sepsis in connexion with uterine cancer is haemorrhage. The infection of cancerous organs by pyogenic micro- organisms makes the labours of surgeons comparable with those of Sisyphus. A careful study of the results published by those surgeons who are making earnest and praiseworthy efforts to relieve, by surgical means, women suffering from cancer of the neck of the uterus, shows that it is not the technical difficulties which baffle, but the difficulty of con- trolling the sepsis. It is this which accounts for the high mortality of what is known as the radical operation for cancer of the cervix ; and among the various micro-organisms which lurk in cancerous tissues the virulent streptococcus is fre- quently found. CHAPTER XXXVIII UTERINE FIBROIDS COMPLICATED WITH CANCER OF THE UTERUS Uterine fibroids are very common, so is cancer of the uterus, and, as the maximum of frequency in relation to age is very nearly the same in the two diseases, it is not a matter for surprise that they should' often co-exist. Whether the presence of fibroids predisposes the uterus to cancer is doubt- ful ; but it may. The subject may be conveniently considered under two headings : — 1. Cancer of the neck of the uterus co-existing with fibroids. 2. Cancer of the body of the uterus complicating fibroids. (Cancer of the Fallopian tubes may be associated with uterine fibroids. See Chap, xxxix.) The subject is an important one, not only as regards treatment, but also from the diagnostic point of view. 1. Cancer of the cervix and fibroids. — The special dan- ger of this combination depends on the fact that it is liable to be overlooked, because the most prominent clinical feature of fibroids, as well as of cancer of the uterus, is bleeding. When a patient with uncomplicated cancer of the neck of the uterus comes under observation, the disease is almost certainly recognized ; but when a woman known to have a fibroid in her uterus complains of more than usual bleeding- she is not so likely to be made the subject of routine exami- nation, hence the disease remains for an indefinite time unsus- pected and therefore undetected. There is also another dan- ger: when cancer attacks the parts around the mouth of the womb its detection is a fairly simple act ; but there is a fair proportion of cases in which the disease begins a short distance up the canal ; such are easily overlooked, and the 393 394 EPITHELIAL TUMOURS higher up the canal the disease is situated the more probable is the chance that it will escape detection, so that if the uterus contains fibroids the chances are very great that the bleeding will be attributed to them, and the existence of cancer will be entirely overlooked. Anyone who follows carefully the published accounts of hysterectomy for fibroids of the uterus, or has had a wide experience of the operation, will learn that a surgeon while performing subtotal hysterectomy examines the cut surface after he has detached the body of the uterus from the cervix, and if it looks suspicious, and he realizes that it is cancerous, the neck of the uterus" is excised. In a few cases subtotal hysterectomy has been performed, and the patient, after recovering from the operation, has had recurrence of the bleeding, and consults the surgeon, who on examination finds that he overlooked a cancerous cervix. On one occa- sion I performed a total hysterectomy, and some months later, as the patient complained of vaginal haemorrhages, I examined her, and found a recurrent cancerous mass occupy- ing the vault of the vagma. The parts removed at the opera- tion had been preserved ; they were examined and a cancer- ous ulceration was found at the os uteri. Although total hysterectomy was performed as a primary operation, in ignor- ance of the existence of cancer, it failed to exercise any in- fluence for good on the progress of the disease. This matter may be summarized thus : It is by no means uncommon for a woman known to have fibroids in her uterus to lead a tolerably comfortable life, in spite of profuse or even long-drawn-out menstrual periods. Occasion- ally a patient of this kind suddenly experiences a marked in- crease in the flow, or has what she terms a "flooding," is alarmed, and seeks advice. Cases of this kind require careful consideration, for this alteration in the syniptoms may indi- cate changes in the fibroid, or the supervention of cancer. If the patient is a spinster, or married but barren, there may be concurrent cancer of the body of the uterus. If married and fertile the co-existence of cancer of the cervix must be con- sidered, and it is well to bear in mind that an early cancer a short distance up the cervical canal will give rise to bleeding and escape detection by the examining finger. There is another aspect of cancer of the uterine neck UTERINE GANGER AND FIBROIDS 395 which must receive consideration. It has been shown that when the body of the uterus has been removed for fibroids, an operation known to surgeons as subtotal hysterectomy, carcinoma has occurred in the cervical stump at such an in- terval after the operation as to make it certain that it did not exist at the time the body of the uterus was removed. Such a case has come under my own observation ; and it has been suggested, especially by Richelot, that it occurs with sufiicient frequency to make it advisable, in operations per- formed for the cure of fibroids, to remove the neck completely with the body of the uterus (total hysterectomy) to avoid such a sequel. This recommendation appears too sw^eeping, especially in view of the fact that even complete excision of the neck of the uterus is not a safeguard against the occur- rence of cancer, for Quenu has reported an observation in which carcinoma arose in the vaginal cicatrix four years and a half after total extirpation of the uterus for disease of the appendages. An instructive record bearing on the subject of uterine fibroids and cancer has been published by Blacker. A woman aged 39 years, with a large uterine fibroid, was submitted to bilateral oophorectomy, and the uterus shrank into the pelvis. Eight years later, carcinoma attacked the neck of the uterus and destroyed the patient. I have had a similar experience. In January, 1902, I removed from the uterus of a woman aged 47 a submucous fibroid by the abdominal route; a right pyosalpinx was re- moved at the same time. She reported herself four years later with extensive cancer of the uterus. 2. Cancer of the body of the uterus complicating fibroids.— This is not an uncommon combination. Cancer of the corporeal endometrium, or, as it is more commonly called in clinical reports, cancer of the body of the uterus, is most frequent at or subsequently to the menopause. The majority of the patients are between the fiftieth and seventieth years : and a large number of the patients are spinsters or harren wives. When a woman complains of irregular uterine bleeding after the menopause an examination is, as a rule, promptly made, and efforts are particularly directed to determine the 396 EPITHELIAL TUMOURS existence or non-existence of cancer. Many women with fibroids do not cease to menstruate, or at least to suffer from a more or less regular loss of blood, for many years after the normal age for the menopause. When cancer of the body of the uterus arises in such a patient it is extremely liable to be overlooked. When a woman known to have a fibroid in her uterus Fig. 191. — Uterus in section, showing primary cancer of the corporeal endometrium associated with fibroids. From a spinster aged 59 years. attains the menopause and remains free from a monthly loss for a few years, then suddenly begins to have "issues of blood," this may be due to cancer of the body of the uterus, and is always such a suspicious circumstance that it demands the most careful examination. The matter may be put in an aphoristic form : Whe^i a woman with uterine fibroids, having passed the menopause, hegins to have irregular profuse uterine hcem,orrhages, it is UTERINE GANCER AND FIBROIDS 397 extreviely probable that she has cancer of the body of the uterus. It occasionally happens that a patient with fibroids may attain her menopause and remain free from losses of blood ; in a few years the fibroid may become infected and bleeding occur profusely as a sequel. It has been suggested, especially by Piquand, that there are reasons for believing that submucous and interstitial fibroids may predispose to cancer of the corporeal endome- trium, for the presence of fibroids sets up chronic metritis,, which renders the endometrium susceptible to malignant transformation. Piquand also analysed a thousand cases of fibroids of the uterus and found cancer of the corporeal endo- metrium present in fifteen ; this is a high proportion. This induced me to examine a consecutive series of five hundred cases in which I had removed the uterus for fibroids, and I found this unhappy combination in eight instances, the nature of the disease in each case being verified by a care- ful microscopic examination. All the patients were over 50 years of age. It is premature to assert that interstitial and submucous fibroids exert such a malign influence as to predispose the corporeal endometrium to cancer ; but it may be true, and it is therefore important to make observations of a clinical and pathological kind, as well as a statistical inquiry, so that a sound judgment may be formed. Blacker, G., " Uterus with Fibroids and Carcinoma of the Cervix," etc. — Traits. Obstet. Soc, 1896, xxxvii. 213. Bland-Sutton, J., " Essays on Hysterectomy," 1905, p. 60 Bland-Sutton, J., " Uterine Fibroids complicated with Cancer of the Body of the Uterus." — Journ. of Obstet. and G^jn. of Brit. Emp., 1906,1. 1. Piquand, " Fibromes et Cancer Uterins." — Ann. de Gyn., Sept. 1905. Quenu, Rev. de Gyn. et. de Chir. Abdom., Sept. -Oct. 1905. Turner, G. Grey, " Cancer of the Cervix and Fibroids." — Brit. Mod. Journ., 1905, ii. 953. CHAPTER XXXIX PAPILLOMA AND CARCINOMA OF THE FALLOPIAN TUBE Papilloma. — Epithelial tumours of an innocent type occur primarily in the Fallopian tube. One of the best-known ex- amples is that recorded by Doran, in which the tube was filled with dendritic masses covered with mucoid fluid (Fig. 192). The ccelomic ostium of the tube was open, and fluid exuded from it into the pelvis. The excrescences grew from all parts of the mucous membrane in the dilated portion of the 192. — Papilloma of the Fallopian tube. {After Boran.) tube. Several pedunculated cysts with thin walls rise from amidst the excrescences and contain papillary outgrowths. The free surfaces of the outgrowths are covered with colum- nar epithelium. Some of the cells bear cilia. The stroma is made up of small fusiform connective-tissue cells, and is poorly supplied with blood-vessels. When a Fallopian tube is stuffed with warts and the ccelomic ostium remains open, the irritating fluid which leaks into the pelvis from the tube causes hydroperitoneum. Doran's case illustrates this fact. A woman aged 50 was 398 GANGER OF THE TUBE 399 repeatedly tapped for ascites and large quantities of fluid were withdrawn. Eventually a tumour was detected in the pelvis ; on removal it proved to be a Fallopian tube stuffed with papillomatous tissue. The patient was in good health twenty-three years later, and the fluid did not reaccumulate in the belly after the operation. When the presence of warts in the Fallopian tube is associated Avith an occluded ccelomic ostium, the fluid cannot leak into the pelvis, but it sometimes finds its way into the uterine cavity and escapes by the vagina. Sometimes a sero- sanguineous fluid escapes in this way in large quantity. Removal of the papillomatous tube arrests the discharge. When warts grow in a tube with an occluded ostium and the fluid cannot run out through the uterine opening, the tube becomes distended into a large banana-shaped cyst, and con- tains chocolate-coloured fluid. In such conditions the warts, which are soft and dendritic, grow most abundantly from that part of the sac which corresponds to the ampulla of the tube. It is a significant fact that papillomatous tubes occur almost exclusively in patients with a history of chronic salpingitis. The faculty possessed by gonorrhoea for producing warts is proverbial. Carcinoma. — Our knowledge of carcinoma of the tube dates from 1888 : now, thanks to the industry of Doran, records of one hundred cases are available for the purpose of guiding surgeons in its detection and treatment. In its leading features this disease simulates cancer of the body of the uterus ; it is most common at and for a few years after the menopause. The chief clinical feature is an irregular blood-stained discharge from the vagina. Primary cancer of the Fallopian tube occurs in women who have had children as well as in those who are sterile. In the greater proportion of patients the disease is unilateral. The symptoms of this disease are so similar to those caused by primary cancer of the body of the uterus, that the cervical canal has been dilated for diagnostic purposes and nothing found within the uterus, but a swelling in the pelvis on one side of the uterus has led to a correct appreciation of the cause of the patient's trouble. Cancer of the Fallopig-n tube sometimes co-exists with 400 EPITHELIAL TUMOURS uterine fibroids. I have met with it on two occasions (Figs. 193 and 194). These two cases are interesting in regard to the efi'ects of treatment. In one woman the mouth of the cancerous tube was open, and in the course of the operation soft growth was seen protruding from it and spreading to the rectum and adjacent peritoneum. I removed much of this soft cancerous material and performed subtotal hysterectomy. BEP J CAU .DEL Fig. 193. — A, Ampulla of a Fallopian tube occupied by a primary cancer ; B, the ampuUa of the tube shown in section. From a sterile married woman 57 years of age. The growth had made its way through the coelomic ostium of the tube and infected the adjacent peritoneum. The patient recovered from the operation and enjoyed good health for eleven months : then sims of recurrence became manifest in the pelvis, and she died a few weeks later. This case illustrates very well the deadly nature of the disease. Examination of a large number of reports testifies that after the removal of a cancerous tube the disease quickly returns and destroys the patient's life. It has been suggested that it would be advantaofeous to remove the uterus as well as the Fallopian tubes. There are, however, other factors to be con- GANGER OF THE TUBE 401 sidered. In another woman with primary cancer of the tube associated with uterine fibroids I removed the uterus as well as the cancerous tube. In this instance the coelomic ostium of the tube was completely occluded and the pelvic peri- toneum remained healthy. The patient recovered from the CANCER Fig. 194. — Fallopian tube with the ovary, mesosalpinx, and adjacent portion of the wall of the uterus. The ostium of the tube is closed and the ampulla distended with a soft cancerous mass, which has extended along the lumen of the tube, and can be traced in its tissues in its course through the uterine wall. The endometrium was not implicated. The uterus contained several large fibroids. operation, and reported herself in good health three years afterwards. This specimen is of great interest, for it appears probable that the closure of the coelomic ostium of the tube when its lumen is stuffed with cancerous material may exercise a great influence in limiting the disease to the tube for a long period, whilst the patency of this orifice will favour the distribution of the cancer within the abdomen. The leakage of cancerous material from the tube into the pelvis is an evil thing, especially Avhen an ovarian cyst is present, for it is very liable to become infected with cancer 2a 402 EPITHELIAL TUMOURS and the condition mistaken for primary cancer ot the ovar}'. On one occasion, when remoTing a large ovarian cyst from a woman 52 years of age, I found the pelvic section of the tumour firmly adherent to the adjacent tissues. On exam- ination of the tumour after removal, the Fallopian tube connected with it had the ampulla stuffed with cancerous rig. 195. — Ovarian cyst infiltrated witli cancer from a primary focus of cancer in the ampulla of the coiTesponding Fallopian tube. material. The ovarian cyst was of the ordinary multilocular type, but the parts in relation with the tube were infiltrated with cancerous tissue, and it could be seen easily that the thick mass on the cyst-wall was continuous with the can- cerous material within the tube. The appearance presented by the parts was as if a stream of cancer-particles had issued from the open coelomic mouth of the tube and implanted themselves on the wall of the ovarian cyst. The patient recovered from the operation, but died a year later with generalized cancer of the abdomen. CANGEB OF THE TUBE 403 Glendining made an interesting observation concerning the spread of carcinoma by the Fallopian tube, founded on a case in which I had removed two ovarian cysts from a woman with well-marked cancer of the stomach. The cancerous particles had infected the cysts, and on microscopic examination they exhibited the characters of implanted cancer (Chap. Li.). To the naked eye the Fal- lopian tube appeared normal, but when it was examined microscopically cancer-particles were found free in its lumen : the subepithelial and plical folds were extensively infiltrated with cancer. From a careful consideration of the matter, Glendining came to the not unreasonable conclusion that infection of the Fallopian tube was brought about by cancer-cells swept into it throusrh its coelomic ostium and ensfrafting' themselves on the mucous membrane, subsequently penetrating to the deeper tissues. It is a noteworthy fact that bilateral cancer is more common in connexion with the Fallopian tubes than in any other paired organ of the body. This supports Glendining's view that many cases of bilateral cancer of these tubes supposed to be primary are really secondary to a focus of cancer in some part of the gastro-intestinal tract. It is also equally possible that a primary cancer in one tube infects the opposite tube through the open ostia. Treatment. — With our present experience it is justifiable to treat primary cancer of the Fallopian tube by operation. In order to give the patients a good chance the tube should be removed as soon as the disease is discovered, and whenever possible the uterus should be removed with it. The operative risks are not so great as when a cancerous uterus is removed, for in the latter case the organ has become septic before its removal is attempted, whereas the isolated position of the Fallopian tube protects it from the invasion of pathogenic micro-organisms : these have easy access to a cancerous endometrium, especially in a multiparous woman. The outlook for patients with primary cancer of the Fallopian tube is sad, because the majority of these women die from recurrence of the disease within a year of operation. 404 EPITHELIAL TUMOUES Bland-Sutton, J., " On Cancer of the Ovary." — Brit. Med. Jonrn., 1908, i. 5. Bland-Sutton, J., " The Clinical Aspect of Secondary Cancer of the Ovary." — Clin. JouTii., 1910, xxxvii. 104. Doran, A., " Papilloma of the Fallopian Tube, associated with Ascites and Pleuritic Effusion."— Trares. Path. Soc, 1880, xxxi. 174. Doran, A., "A Table of over fifty complete Cases of Primary Cancer of the Fallopian Tube."' — Joxi,Tn. of Oistet. and 6yn. of the Brit. Emjp., 19C4, vi. 285. Doran, A., "Primary Cancer of the Fallopian Tube." — Ihid.. 1910, xvii. 1. Glendining, B., "The Spread of Carcinoma by the Fallopian Tube." — Arcli. of Middx. Hosp., xix. 82. GROUP IV. ENDOTHELIAL TUMOURS CHAPTER XL ENDOTHELIOMAS The peculiar flattened cells known as endothelium which line the interior of blood-vessels and lymphatics, and cover the surface of serous membranes such as the pleura, pericardium, and peritoneum, are the source of malignant tumours known as endotheliomas. In their general characters endotheliomas resemble the carcinomas and sarcomas, but, though decidedly malignant, they run a slower course than the typical sarcomas. Endotheliomas which arise in blood-vessels are termed hsemendotheliomas ; those starting from lymph- vessels are lymphendotheliomas. There is also a variety which arises in the perivascular lymphatics, known as peritheliomas. Some of the most characteristic examples of hsemendo- theliomas may be regarded as malignant angeiomas, and of lymphendotheliomas as malignant lymphangeiomas. Some of the best examples of endotheliomas grow in connexion with the gums, where they resemble in their general features and microscopic structure the cystic epithelial tumours of the jaws {see p. 212). An important set of tumours which belongs to this group, and which has long been a pathological puzzle, is the so-called sarcomas of the salivary glands, especially those which grow in the parotid gland. These tumours appear as oval, smooth, elastic swellings, which burrow deeply into the gland, dip beneath the sterno-mastoid, and acquire attachments to the sheath of the carotid artery and internal jugular vein. The facial nerve is usually involved in large tumours of the parotid, and is liable to be injured when attempts are made to remove them. 405 406 ENDOTHELIAL TUMOURS When left to themselves sucli tumours cause death in a variety of ways. Thus they may press upon the pharjaix and lead to fatal dysphagia, or ulceration may open some A V-..i, Yin /ir' ;r", iii^iir iiim rf * I. mM^m Hi Fig. 196. — ^Parotid tiimour whicli had been slowly growing seventeen years. When the woman was 57 it grew rapidly and infected the lymph-glands, and destroyed the patient in six months. large vessel in the neck and produce fatal haemorrhage; secondaiy nodules sometimes form in the lungs, and induce fatal broncho-pneumonia. ENDO TEELIOMAS 407 Structurally, these tumours exhibit extraordinary variety. Some consist entirely of hyalin cartilage arranged in lobules bound together by loose connective tissue. The cells of the cartilage rarely possess capsules, and are often stellate, as in immature cartilage. Such tumours otow with extreme slowness, and rarely exceed a bantam's egg in size ; Fig. 197.— Chondroma of the submaxillary glaud which had been slowly growing forty-four years. It was successfully removed. they may require ten or even twelve years to attain such proportions. The large, rapidly growing tumours consist of spindle cells in which tracts and islets of hyalin cartilage are in- terspersed. When chondral tissue is abundant, it is very prone to mucoid changes, and soft, fluctuating spaces are formed. The connective tissue is very liable to undergo myxomatous change, and, as if to render these tumours 408 ENDOTHELIAL TUMOURS more complex, portions of the secreting tissue of the gland are imprisoned in them. It is not unusual in sections from a parotid sarcoma to meet with spindle cells, cartilage, myxomatous tissue, glandular acini, and fibrous tissue in an area of 2 cm. square. Exceptionally, transversely striped spindle cells are seen. Parotid tumours of such complex structure grow rapidly and attain a large size, and often infiltrate the Fig. 198. — Microscopic appearances of a hsemendothelioma of the kidney. (After Ziegler.) surrounding tissue and skin. Some of them infect the adjacent lymph-glands (Fig. 196) and give rise to secondary deposits in the lungs. Chondrifying tumours of the parotid are most frequent between the fifteenth and thirty-fifth years, but they have been observed as late as the seventy-fourth year. In their early stages they are easily removed, but many of the rapidly growing forms so quickly infiltrate the surrounding parts that their complete extirpation is not always possible. Endotheliomas are far less frequent in the submaxillary than in the parotid gland (Fig. 197). They are encapsuled and, ENDOTHELIOMAS 409 as a rule, shell out easily. They grow slowly, and occur in the young as well as in adults. Glandular tissue is often associated with the cartilage. Similar tumours occur in the lachrymal gland ; they are extremely rare in this situa- tion. The chief features of endotheliomas in the parotid gland are these : a tumour will arise in the gland and Pig. 199. — A breast in section ; it contains an endothelioma (perithelioma) . The nipple is unaffected. grow to the size of a walnut, and remain stationary ten, fifteen, and even forty years; then without warning it enlarges, infiltrates the gland, causes pain, and destroys life in a few months. In another case the tumour will arise, grow quickly, ulcerate, and destroy the patient's life in six or nine months. The microscopic structure of the tumours is similar. Endotheliomas have been observed in the mammary 410 ENDOTHELIAL TUMOURS gland, in the skin (especially in association witli moles and warts), the uterus, and the omentum. These tumours some- times present clinical characters by which they may be recognized. For example, a woman came under observation with a globular tumour in her breast. She stated that the tumour had been growing slowly for two years. The skin covering the mass was smooth and not infiltrated b}'' growth, and the nipple projected normally. The axillary lymph- nodes were enlarged. The characters of the tumour differed from those usually presented by primary mammar}" cancer. Fig. 200.— Microscopic characters of a perithelioma, showing the cell-mantle around the blood-vessels. (After Ziegler.) The breast was amputated, and on microscopic examination the tumour proved to be a perithelioma (Figs. 199 and 200). Peritheliomas. — This variety includes the rare tumour styled angeio-sarcoma (Ziegler). On microscopic examination the tissues of such tumours resemble superficially the lobes of the hver; this peculiarity depends on an overgrowth of cells in the perivascular sheaths of the small vessels (Fig. 200). Psammomas (dura-endotheliomas). — This variety grows in connexion with the dura and the pia mater of the brain and spinal cord. These tumours are called psammomas, or sand tumours, on account of the presence in varying quantity of earthy matter like that in the pineal body. Cholesterin is also present, and often in such quantity that the tumour PSAMM0MA8 411 assumes a pearly lustre when exposed to light. A very remarkable example of this is preserved in the museum of the Royal College of Surgeons, London. The tumour, which fills the fourth ventricle, has an average diameter of 10 cm.; it Fig. 201. — Psammoma lying in relation with the flocculus. The patient, a man aged 36 years, died from an attempt made to remove the tumoui'. The chief symptoms were deafness, pain, vomiting, giddiness, and nystagmus. looks like a solid pearl disparting the two halves of the cere- bellum, and projects between the inferior vermiform process and the medulla. The catalogue contains an interesting clinical history furnished by Miss B. Knowles. A psammoma rarely exceeds in size a shelled walnut, and when growing in connexion with the choroid plexuses of the 412 ENDOTHELIAL TUMOURS cerebral ventricles these tumours may be bilateral ; when large they form deep bays in the adjacent brain-tissue, and when growing in the immediate vicinity of important nerves cause severe and disastrous consequences (Fig. 201). The structure of a typical psammoma (Fig. 202) shows its intimate relation to blood-vessel ; each concentric body has a vessel for its centre. A common place for a psammoma is the immediate neigh- bourhood of the flocculus. In this part of the cranial cavity they are often bilateral, and for many years I have believed i "^^N*^ //^w/ /'>-..> W^^ " III' Fig. 202. — Microscoijical appearance of a typical psammoma. that in this situation they arise from the processes of cho- rionic villi belonging to the fourth ventricle which at this spot emerge from the cornucopia. It is easy to understand that tumours growing in close relation with such important nerves as the trigeminal, facial, vagus, would soon lead to symptoms and surely attract at- tention, and, as a matter of fact, a large number of examples have been recorded under a variety of names, such as sar- comatous tumours of the fifth and seventh nerves ; fibro- sarcomatous tumours of the flocculus ; symmetrical tumours of the medulla, and the like. A man with bilateral tumours of this kind was violent, blind, deaf, and suicidal (Strahan). In another case a psam- PSAMMOMAS 413 moma measuring 7'5 by 6 cm., growing from tlie membranes immediately covering the median lobe of the cerebellum in a lad, caused headache, vomiting, blindness, optic neuritis, priapism, opisthotonos, and other disturbances, ending in death (Beevor). Psammomas of the spiral membranes are as dangerous when seated high in the spinal canal as psammomas near Pia mater Fig. 203. — Portion of the spinal cord with a psammoma situated at the level of the intervertebral disc between the tenth and eleventh thoracic vertebrse. From a woman 46 years of age. {Museum, Middlesex Hospital.) the flocculus. In the spinal canal these tumours do not attain a large size — indeed, in the few recorded cases there is singular uniformity in their shape and dimensions (Fig. 203). Treatment. — Psammomas of the spinal membranes have been successfully removed by surgeons. In the cranial cavity the accurate diagnosis and localization of such tumours has been accomplished ; they have also been removed, even when lying in the vicinity of the flocculus, in spite of their sub- tentorial situation, but rarely with success. Psammoma-bodies are fairly common in the choroid 414 ENDOTHELIAL TUMOURS plexuses of the lateral ventricles of horses. When the tumours are large they produce grave and even furious symptoms. In some of the reported cases, horses have destroyed themselves by wild plunges made in attacks of delirium. These tumours are very vascular; some are soft, others are hard. Nearly all contain cholesterin. Probably they are of inflammatory origin. Beevor, C. E., "A Case of Tumour of the Cerebellum."— i?ram, 1881-2, iv. 250. Nash, W. G., " Primary Sarcoma of the Omentum." — Proc. Roy. Soc. of Med., Obstet. Sec, 1909, ii. 226. Strahan, " Symmetrical Tumours at the Base of the Brain." — Journ, Mental Sou, 1884, xxix. 246. GROUP V. TUMOURS ARISING FROM THE CHORIONIC VILLI CHAPTER XLI CHORION -EPITHELIOMA (DECIDUOMA) In 1889 Sanger and Pfeiffer independently described a variety of malignant disease arising in the uterus which presented microscopic characters so strongly resembling decidual tissue that the disease was named decidiioma ')nalignum. Sub- sequent investigations by other observers brought to light the important fact that this remarkable disease is very liable to arise in the endometrium within a few weeks or months of abortion, or of delivery at term, and especially after the expulsion of the so-called "hydatid mole." Moreover, the microscopic investigation of the tumour showed that it conformed in histologic type to the multinuclear mantle or syncytium which covers the chorionic villus. This discovery led to a change of opinion as to the source of the disease, and as most writers regard it as arising from changes in the epithelial elements of the chorionic villi rather than in the decidua, the name chorion-epithelioma has come to be adopted in preference to deciduoma. Before considering the essential features of this disease the change in the chorion known as the hydatid mole needs a brief description. The normal villi of the chorion in the early stages of their development consist of an axis or core of delicate connective tissue covered with epithelium arranged in two layers: the inner is known as Langhans layer; the outer, called the syncytium, is peculiar, and resembles a large elongated multi- nucleated cell enveloping the villus like a mantle. In the early stages, the connective-tissue core of the villus is devoid of blood-vessels : the tissue in these early stages consists of 415 416 OHOBION-EPITHELIOMA brandling cells separated from eacli other by mucoid inter- cellular substance ; later, the cells become spindle-shaped and the tissue denser and vascularized. In the disease known as hydatid mole the villi become changed into transparent grape-like bodies (Fig. 204), and look not unlike the vesicles so characteristic of the cystic stage of Tania echinococcus (hydatids), and a hundred years ago the grape-like bodies or vesicular bodies were regarded as parasites, especially as the embryo is rarely to be found in these specimens. Fig. 204.— Hydatid mole. (After Bumm.) In 1827 Madame Boivin and Velpeau showed that the disease depended on a change in the chorionic villi. Yirchow gave attention to the histology of these vesicle-like bodies, and considered them to be due to a myxomatous change in the villi (1853). This view prevailed until Marchand in 1895 demonstrated that the essential feature of the change depends more on the epithelium than on the stroma of the villus, for it undergoes irregular proliferation and assumes invasive characters, penetrating the decidua and even the muscular wall of the uterus. The vessels of the villi dis- appear, the stroma degenerates, and the swollen condition of the so-called vesicles is the result of cedema rather than of HYDATID HOLES 417 mucoid change. The mvasiveness or destructiveness of these altered villi has long been recognized, and specimens have been observed in which the villi have perforated the uterus and caused fatal bleeding into the abdominal cavity. The hydatid mole (or chorion-ejnthelioma benignum) is not common ; it has been estimated by one writer (Madame Boivin, 1827j to occur once in 20,000 pregnancies, and by another (Williamson, 1899) once in 2,400. It is quite certain that only a small proportion of women who have expelled hydatid moles suffer from chorion-epithelioma, but no reliable Fig. 205. — Microscopic appearance of a chorionic villus from a hydatid mole, in transverse section. estimates are available. The liability of a woman who has had a miscarriage of this kind, to be the victim of such a deadly disease as chorion-epithelioma malignum, renders it advisable that she should keep under medical supervision for some months after such an event. Some writers are disposed to believe that there are two varieties of the hydatidiform mole, one being purely innocent, the other giving rise to the malignant chorion-epithelioma. As yet microscopical inquiries have not provided these theoretical distinctions with a histologic foundation. Relation of the hydatid mole (chorion-epitheliotna benignum) to lutein cysts. — Some valuable observations have been made 2 B 418 GHOBION-EPITHELIOMA on the frequent association of bilateral lutein cysts of the ovary and the so-called hydatid mole ; indeed, the presence of lutein cysts in this disease is constant enough to lead to the belief that the two conditions are correlated. The lutein cysts are large enough to be of clinical importance, and they have been known to obstruct delivery and in one instance to cause acute symptoms by undergoing axial rotation. This has given a new interest to the yellow tissue which composes the greater part of a corpus luteum, and some observers state that it furnishes an internal secretion, and that the adhesion of the oosperm to the endometrium depends on a proper supply of this hypothetical fluid. Fig. 206. — Portion of a chorionic villus from a hydatid mole more highly magnified and showing a piece of decidua. Fraenkel has elaborated this theory, and his views receive the support of some competent German pathologists ; an over- production of this secretion, the result of a plus quantity of lutein tissue, sets up, according to Pick, a " chorion-epithe- liomatous reaction " in the embedded ovum and leads to the formation of a benign chorion-epithelioma (hydatid mole), Lockyer has made a careful study of this question, and the result of his painstaking inquiry lends great support to the view that there is a close correlation between lutein cysts and chorion-epithelioma of both kinds. GHOBION-EPI THELIOMA 419 Chorion - epithelioma malignum (deciduoma). — The uterus when attacked by this disease usually enlarges and often becomes big enough to be appreciable as a tumour in the hypogastrium : its contour may be nodular. In some patients the disease is limited to the endometrium, and the primary focus of the disease may be so small as not to cause enlarge- m.ent of the uterus. Some very exceptional cases have been described in which the disease did not involve the uterus, but began in the vagina. Fig. 207. — MiCioscopic uhaiacteis of a i.ell-maoo fiom a cliOiioii-cpitLelioma showing large decidua-like elements, and the forms intermediate between the Langhans' layer and the syncytium. {After John H. Teacher.) The intimate dependence of chorion-epithelioma on changes associated with pregnancy is illustrated by the fact that the disease occurs primarily in the Fallopian tube as a sequel of tubal pregnancy. {8ee Risel.) The result of the examination of a large number of examples of this disease by many investigators has established the fact that it arises in portions of the chorionic villi which remain embedded in the endometrium after the expulsion of 420 CHORION-EPITHELIOMA the main products of gestation, and especially if the villi have undergone hydatidiform change. Some competent authorities still believe that there may be two varieties of this disease, one arising from the epithelial elements of the chorionic villi and the other in decidual tissue. To the naked eye the tumour-tissue appears on section as a soft reddish mass. " Histologically a chorion-epithelioma consists of well-defiued cells of various shapes and sizes Fig. 208. — Portion of a clioriouic villus from a chorion- epithelioma, showing the origin of the tiunour from the epithelium of the villi. {After John M. Teacher.) closely packed together, and large multinuclear irregular masses of protoplasm in which no definite cell-masses are recognizable. This tissue invades and destroys the uterine tissues after the manner of a malignant growth. It contains no proper connective-tissue stroma, or blood-vessels of its own." (Teacher.) A remarkable feature connected with chorion- epithelioma is the discovery that some teratomas of the thorax and of the testis contain tissue indistinguishable from that of chorion-epithelioma. The eroding power of the cells of a chorion-epithelioma enables them to penetrate the tissues and gain entrance to CHOEION-EPITHELIOMA 421 veins; fragments are deported by the blood-stream to lodge in lungs, bones, and other viscera, and grow into fjecondary deposits. The common situations for these deposits are the lungs and vaginal veins. The course of the disease is marked by oft-recurring profuse bleeding from the uterus ; rigors ; pyrexia ; great emaciation and the signs of dissemination, such as secondary nodules in the lungs, bones, and the abdominal viscera. The disease is fatal, and runs usually a very rapid course, but it exhibits remarkable variations in virulence : the view is held by some observers that the virulence is greater after an abortion than when it supervenes on a pregnancy which has run to term or after the expulsion of a hydatid mole. The chief clinical signs are frequent bleeding from the uterus, producing great an£emia, and accompanied usually by enlargement of the uterus following a recent labour or miscarriage. Many of these signs are caused also by the retention of a fragment of placenta, or a uterine mole. In such circumstances the cervical canal should be dilated and the cavity of the uterus explored ; any retained fragments of conception that are removed should be submitted to careful microscopic examination in order to establish a reliable diagnosis. Treatment. — The most satisfactory method of dealing with this disease is prompt removal of the uterus. Teacher considers it reasonable to conclude that operation offers a fair chance of recovery, and that it may be done with some prospect of success in the face of the gravest signs of disease and even if metastasis has occurred. An admirable summary of knowledge relating to lutein cysts and their relation to chorion-epithelioma (hydatid mole) is given by Cuthbert Lockyer. — Joicrn. of Obstet. and Gyn. of Brit. Emp., 1905, vii. 1. An admirable summary relating to chorion - epithelioma and a complete catalogue of the literature is furnished by John H. Teacher. — Trans. Obstet. Soc, London, 1903, xlv. 256. Risel, " Zur Kenntniss des Primaren Chorionepithelioms der Tube." — Zeitsclir. f. Gcb. unci Gj/n., 1905, Ivi. 155. GROUP VL TERATOMAS AND DERMOIDS CHAPTER XLII TERATOMAS In this group we have to consider three remarkable genera of tumours which in their type-forms are as easily distinguished as a butterfly and a buttercup, yet examples occur presenting such composite characters that it is difficult to assign them to a particular genus. The difficulty in regard to such compound tumours as teratomas and dermoids occurs especially in relation with the male and female genital glands. There are two forms of teratomas, external and internal. This chapter will be devoted to external teratomas. A teratoma is an irregular conglomerate mass containing the tissues and fragments of viscera belonging to a sujypressecl fcetus attached to an otherivise normal individual. EXTERNAL TERATOMAS In order to appreciate the nature ol these singular mal- formations it will be necessary to consider the subject of con- joined twins, supernumerary limbs, and acardiac foetuses. In the animal and vegetable kingdom it occasionally happens that a single ovum gives origin to two embryos, which may be quite separate from each other (twins), or they may be united, a condition known as conjoined twins (Fig. 209). When twins arise from a single ovum they are said to be uniovular, and as they are invariably of the same sex they are termed homologous. Conjoined twins are always homolo- gous and uniovular. When two embryos are conjoined, and one goes on to complete development, whilst only certain parts of its com- panion continue to grow, the result is a parasitic foetus. The mature individual supporting it is called the autosite. 422 CONJOINED TWINS 423 In other examples the suppressed foetus consists of an irregular-shaped tumour growing, perhaps, from the posterior surface of the sacrum, or within the abdomen or thorax, which on dissection contains a fcAV vertebroe, or processes of Fig. 209. — The conjoined twin-sisters Eadica and Doodica at the age of 3J years; born in 1889 at Noapara, a village in the province of Orissa, India. In 1899 they were re-exhibited in London in excellent health. Doodica died in 1902 {see p. 432). skin resembling digits, associated with a piece of intestine or an imperfect liver. This is a teratoma. In order to demonstrate the relation between parasitic foetuses and teratomas, it will be useful to refer to dichotomy. In animals and vegetables there is a strong tendency for parts ending in free extremities to bifurcate or dichoto- mize. When this affects digits the result is supernumerary 424 TERATOMAS fingers and toes. Should it extend to the axis of the Hmb, supernumerary legs, wings, or fins are produced. Dichotomy is not confined to the limbs, but affects also the axis of the trunk. When the whole embrj^onic axis dichotomizes, twins are produced. Should cleavage be partial, and affect the caudal end of the trunk, it is spoken of as posterior dicho- tomy. When it involves the anterior end it is called anterior dichotomy. With complete dichotomy, in which both era- Fig. 210. — Posterior view of J._B. clos Santos at the age of sixmonths. {After Acion.) bryos go on to full development, either as separate or con- joined twins, we are not further concerned, and considera- tion of the conditions arising from the imperfect growth of one embryo whilst its companion continues to develop must be deferred until we have discussed the results of partial dichotomy. Posterior dichotomy. — When cleavage involves the caudal section of the trunk-axis to any serious extent it necessarily follows that the pelvis as well as the vertebral column will be reduplicated; it is also obvious that the reduplication of the pelvis involves a corresponding increase in the number of the pelvic organs, including the limbs. Thus it follows that supernumerary hind limbs may arise from dichotomy SAGBAL TERATOMAS 425 affecting the embryonic limb, or from cleavage of the caudal end of the trunk. The two modes also hold good for redupli- cation of the fore limbs. The limbs may project from the ventral aspect of the pelvis, or be, as it were, dislocated on to the dorsal surface. Occasionally they occupy a position mid- way between these two extremes and lie more or less parallel with the normal hind limbs. In some individuals one pair of supernumerary limbs fuse throughout their length (Fig. 210), and in others one limb is --^ DIMPLE Fig. 211. — Sacral teratoma with a suj)emumerary leg. suppressed (Fig. 211), but it is a noteworthy fact in its bearing on the cleavage theory that in all specimens of super- numerary limbs due to posterior cleavage there is an accessary but usually imperforate anus. In the case of Jean Battista dos Santos of Portugal, described in 1846 by W. Acton, and nineteen years later by Ernest Hart in London, and by Handyside in Edinburgh, there was not only an additional (imperforate) anus, but the man had two functional penes. It is also an interesting fact that malformed individuals of this kind, whether male or female, are capable of producing healthy, well-formed offspring, the most striking example being the Siamese twins, Chang and Eng Bunker. They married sisters : Chang had ten children, Encf twelve. One boy and one girl of Chang's were deaf and dumb; there 426 TERATOMAS was no other blemish in the famih'es of the twins. The pygopagus twins, Rosa-Josepha Blazek, when 32 years of age conceived and brought forth a hving son at Prague. The child was in Rosa's womb, but milk appeared in the breasts of both (Trunecek). Duplication of the pelvic limbs and of the anus occurs frequently in sheep, calves, and birds. When the parasitic foetus is so suppressed as to form only Fig. 212. — Anterior dichotomy. om a photograph supplied hy Dr. William Bitcld, of Bristol, July 2(jth, 1856, to Sir James Paget.) a shapeless or deformed lump, such as would be the case in Fig. 211 if the limb were absent, then the mass would be called a teratoma. It is a fact that the autosite has no power of initiating in- dependent movements in the limbs of the parasite, neverthe- less he can localize . the prick of a pin on the parasite, and feel uncomfortable when it is cold. In the parasitic fcetus represented in Fig. 214, micturition occurs independently CRANIAL TERATOMAS 427 and without the knowledge of the autosite until he feels urine trickling over him. Involuntary twitchings can some- times be induced in teratomas by irritating them. Anterior dichotomy. — Cleavage may affect the facial portion only and produce reduplication of the jaws, or it may involve the head and produce a two-headed individual. Should it extend to the thoracic region of the spine, then an animal with two heads and reduplicated fore-limbs is the result. When partial dichotomy attacks the head the median parts of the reduplicated face are so conjoined and malformed that they are sometimes found hanging in the pharynx, being attached to its roof by a pedicle. Such tumours are called hasicranial teratomas : the majority of tumours called pharyngeal dermoids are of this nature. In order to appreciate the difficulty of interpreting the nature of tumours covered with skin and bearing teeth, reference should be made to the section on Heterotopic Teeth (Chap. liv.). It is curious to find in a teratoma an organ like a vertebra, or a tooth, or a tongue well developed, although the rest of the fcetus is represented by a mere conglomeration of tissue. Among remarkable instances of anterior dichotomy, Millie- Christine, the blended Tocci brothers, and Rosa-Josepha have been carefully described. This form of dichotomy has been studied in fishes, tortoises, calves, birds, and snakes. The details of the remarkable child represented in Fig. 212 were supplied to Sir James Paget by Dr. William Budd, of Bristol, in 1856. With the exception of the extraordinary excrescence, he writes, " the child presents no deviation from the normal type, but is as comely a little thing as you would wish to see. Every movement and every act of the natural face is simultaneously repeated in the second face in the most perfectly consensual manner. When the natural face sucks the second mouth sucks." Crying and yawning occurred at the same time in the two faces. I have ventured to publish this case because, so far as my knowledge of teratology extends, no similar case in the human subject is known. The fact "that every movement and every act of the natural face is simultaneously repeated in the second face in the most perfectly consensual manner " 428 TERATOMAS is quite in accord with what has been observed in calves the subject of " partial anterior dichotomy." Thus far we have been concerned with duplicated parts that reach such a standard of development that their identifi- cation is a matter neither of difficulty nor of doubt. It will now be necessary to consider the meaning of those attached parts named j)arasitic foetuses, and the irregular masses called teratomas. It happens, and not infrequently, that in cases of twins . one oi them is of natural shape and viable, but the other is very imperfectly developed, and as it lacks a heart (or if this organ be present it is rudimentary and functionless) it is said to be acardiac. The degree of development varies greatly : in Tulaercle mark- ing the end of the rudimentary sxjinal cord. \i i.rdiac fcetus. some the foetus may be complete save head and neck. In rarer cases the foetus may be merely represented by an irregular-shaped mass consisting of oedematous integument surrounding a portion of the skeleton, usually an innominate bone with the bonj^ elements of a lower limb. In some specimens no particular skeletal element is recog- nizable, but a portion of intestine or rudiments of the genito- urinary organs can be detected. To such examples of acar- diacus the adjective amorphous is applied, and to French teratologists they are known as " anidian monsters." An acardiac such as Figc. 213 has been described as a dermoid of AGABDIAGS 429 the umbilical cord (Budin). In very exceptional cases the acardiac may be so thoroughly amorphous that it is impos- sible to decide its nature until it has been submitted to a microscopic examination (Lea). Acardiacs are not necessarily separate from the well- developed twin, but may be attached to it in a variety of ways. Fig. 214. — Laloo, a Hindoo, with an acardiac parasite attached to his thorax. In the common form the shapeless mass is connected with the dorsal aspect of the sacrum, and simulates a spina bifida sac, or the form of congenital sacro-coccygeal tumour which arises in the postanal gut. These sacral teratomas often twitch when irritated, and this is a valuable diasrnostic sicfn. In rarer cases teratomas have been observed in the thoracic and abdominal cavities connected with the vertebral column. 430 TERATOMAS They are not uncommon on the head, particularly in relation with the jaws (p. 435). The explanation of acardiac foetuses, whether free or para- sitic, seems to be this : Two embryos arise from a single ovum ; in some instances the cleavage is complete, but the heart of one embryo is defective. The circulation of the two embryos is continuous at the placenta, and the heart of the normal embryo is able to maintain in a measure the blood- current in its companion, and thus save it from complete Placenta of the acardiac. Artery and vein dis- tributing blood to the acardiac. Umbilical cord of the healtliy twin. ' Fig. 215. — Placenta from twins, one of which was an acardiac. (After Astley Cooper.) suppression. Sir Astley Cooper demonstrated this compen- satory mechanism in the case of an acardiacus placed in his hands by Dr. Hodgkin. An inspection of the drawing of the placenta from this case (Fig. 215) shows that the umbilical vessels in the two sections of the compound placenta were directly continuous. In the case of a parasitic acardiac — e.g. Laloo — the circula- tion must be directly maintained by the heart of the autosite, as an independent heart has not, so far as I am aware, been detected in the parasite. The blood-current is always ex- tremely sloAV in the acardiac, and thermometric observations FAEASITIG FCETUS 431 demonstrate that its temperature is several degrees lower than that of the autosite. Thus a study of the circumstances surrounding the de- velopment of twins and duplex monsters brings us to the conclusion that a teratoma ma}'' arise either from partial dichotomy of the trunk-axis of the embryo or from complete dichotomy. In the latter case, while one twin has gone on to full development, the growth of the other has been arrested, and in some cases the suppression has been so great that the Fig. 216.— Sacred ox with a parasitic calf (India). companion foetus is represented by a deformed or shapeless mass consisting of integument covering ill-formed pieces of the skeleton and portions of viscera. The best evidence that parasitic foetuses and teratomas arise from cleavage is this : we always find hke parts attached to hke parts — head to head, pelvis to pelvis, thorax to thorax. To this I do not know an exception. Treatment. — Parasitic acardiacs and conjoined twins are so valuable as sources of gain in fairs, shows, and large 432 TERATOMAS cities that the parents, or the unscrupulous individuals who get possession of these children, will not permit operative interference. The xiphopagous twins Radica and Doodica (Fig. 209) are remarkable in this respect, for Doodica became the victim of tuberculous peritonitis and Doyen divided the uniting band. Doodica died six hours after the operation, but Radica survived (1902). In the AVest the parasitic foetus is a source of unholy gain ; among Hindoos it is an object of veneration, especially when the autosite is a cow. Acton, W., " An account of a Case of Partial Double Monstrosity." — Med.- Chir. Trans., 1846j xxix. 103. Budin, P., " Note sur une Tumeur da Cordon Ombilical." — Pror/res Med., 1887, V. 550. Geoffrey Saint-Hilaire, Isidore. — "Anomalies de I'Organisation," 1836, iii. 166. Handyside, P. D., " Observations on the Arrested Twin Development of Jean Eattista dos Santos, born at Faro in Portugal, in 1846." — Edin. Med. and Surg. Journ., 1866, ii. 833. Harris, R. P., " The Blended Tocci Brothers and their Historical Analogues." — Amer. Journ. of Obstet.. 1892, sxv. 460. Hart, Ernest, '■ A Remarkable Case of Double Monstrosity in an Adult." — Lancet, 1865, ii. 124. Hodgkin, T., and Cooper, Sir A., " The History of an unusually formed Placenta and imperfect Foetus, and of similar examples of monstrous productions." — Guy's Hasp. Repts., 1836, i. 218. Keith, A., " The Anatomy and Nature of two Acardiac Acephalic Foetuses." — Trans. Obstet. Soc, 1901, xlii. 99. Lea, A. W. W., "Report of Committee on Dr. Arnold W. W. Lea's specimen of Tumour expelled from Uterus during labour at term, exhibited January 4th, 1899."— I'mHs. Obstet. Soc, 1900. xli. 219. Trunecek, C, " L'Accouchemect du Pygopage Rosa-Josepha Blazek."— Zis Se/naine Med., 1910, No. xx. 299. Windle, B. C. A., " On the Condition known as 'Epignathus."—Jour7i. Anat. and Phijs., 1898-99, xxxiii. 277. CHAPTER XLIII TERATOMAS (Concluded) INTERNAL TERATOMAS This variety occurs in the thorax, the abdomen, and the cranium ; in the abdomen it occasionally attains a degree of development equal to that found in external parts. The internal teratoma differs from the external kind in being enclosed in a cyst, and it imperils the life of the autosite from mechanical causes, and in rare instances by displaying malignancy of a remarkable kind. It is unusual in these cavities of the body to find teratomas with limbs and organs so shaped as to enable the observer at once to recognize that he has before him a very badly developed embryo enclosed within its bearer, and it is customary to denominate such conglomerate lumps teratoid tumours. Intra-abdominal teratomas. — A parasitic foetus within the abdominal cavity is extremely rare; one of the best-known examples was described by Young in 1808, under the title of "A Foetus found in the Abdomen of a Boy." In this instance a large cyst was found in the belly of an infant a year old. The post-mortem examination was carefully conducted, and the cyst, which lay behind the peritoneum, contained, in addition to a large quantity of fluid, the pelvis, lower limbs, and genital organs of a foetus (Fig. 217). Five years later Phillips described, in a letter to Sir Benjamin Brodie, a case in which parts of a foetus were found in a tumour lodged in the abdomen of a girl 2| years of age. The brief description contains this statement : " The cyst in the abdomen contained fluid and solid matter ; the latter contained a large bone resembling a tibia covered with muscle, and small bones like a tarsus. There were cystic spaces containing sanious fluid. The liver bore marks of inflammation and w\as studded with tubercles." 2 c 433 434 TERATOMAS Lexer lias described a teratoma as big as a fist removed during life from a girl 7 weeks old ; it was situated in the foramen epiploicum and lay under the liver. This tumour had cystic and solid parts ; the latter represented skeletal and visceral elements. The baby did not survive the operation. Intrathoracic teratomas. — Tumours described as derm- oids within the thorax have been recorded by many writers. They are rare, but cause much distress to the patients who possess them. The majority occupy the mediastinum and Fig. 217- — ^A foetus which was found enveloped in a cyst in the abdomen of a hoy. (After Youinj, 1808.) grow downwards to one or other side, compressing the lung. A dermoid has been observed anteriorly to the pericardium (Hale White). Many of the cases have been recorded as " dermoids of the lungs," but all the later reporters agree that the involve- ment of the lung is secondary. When the bronchi become implicated by such a tumour, "hair-spitting" occurs, due to the cyst opening into the air-passage as a consequence of INTBAGBANIAL TERATOMAS 435 suppuration. The inner wall of such cysts is often beset with nipple-like processes of skin. Ritchie has described a teratoma which occupied the mediastinum of a man of 24 years : attached to and forming part of its wall was a solid tumour containing tissue micro- scopically identical with that of a chorion-epithelioma. The lungs and liver contained secondary deposits. It is somewhat remarkable to lind among such highly organized tumours, whose extreme specialization would almost pass as a brand of innocency, illustrations of what has already been mentioned in connexion with other Groups, that each genus of the so-called benign tumours contains varieties which .shade away indefinitel}^ from the type species and display malignancy. Intracranial teratomas. — In the chapters dealing with sequestration dermoids it is pointed out that these tumours are found in connexion with the scalp and in association with the tentorium, and their presence in these situations may be attributed to small portions of surface epiblast sequestered in the course of the development of the skull (p. 461). Such dermoids exhibit the same characters as those so commonly found near the angles of the orbits (p. 455). Complex tumours of the teratoid type are occasionally found at the base of the skull, and usually occupying the pituitary fossa. Teratomas in this situation resemble those found at times in the pharynx, and contain striped-muscle fibre, hyalin cartilage, glandular tissue, and cysts lined with squamous epithelium. In one carefully described specimen ganglion-cells and white nerve-fibres were present : some of the nerve-bundles had a cross section as big as the radial nerve. Pituitary teratomas have been described by Lawson, Bowlby, Hale White, Sainsbury, Buzzard, and Bostroem. Rows de- scribed two examples which occurred in men ; one was aged 77 and the other 73. Intracranial dermoids or embryomas occur in the basal parts of the brain, in or near the middle line. They grow very slowly, and rarely produce symptoms. Teratomas of the pharynx and palate. — It is noteworthy that the parts in relation with the cephalic as well as the caudal extremity of the notochord are common situations for teratomas containinsr formed orsfans and tissues such as bone, skin, striped muscle, nerves, epithelium, and occasionally a 436 TERATOMAS tooth, but devoid of any shape and arrangement of the parts to suggest a foetus, though arising in the same manner as a parasitic foetus. In the palate and naso-pharynx, teratomas usually take the form of pedunculated tumours clad with skin which is often pilose (Fig. 218). The core of these tumours consists of connective tissue which may contain hyalin cartilage and a variable amount of striped muscle- tissue. In many cases it is difficult to decide whether the tumour grows from the palate, or from the base of the skull and projects through a gap in the bony palate. Sometimes the attachment is so slender that the tumour undergoes Fi^. 218. — Pedunculated skin-clad pilose tumour from the pharyngeal aspect of the soft palate. {A riiold. ) spontaneous detachment ; in the case reported by Lambl the child, swallowed the tumour and voided it next day by the anus. Occasionally the tumour is sessile, and may even project into the floor of the pituitary fossa and compress the optic nerves and tracts. Windle has collected the literature relating to teratomas of the pharynx under the title of Epignathus, and has sum- marized the various views in regard to the nature of this condition. In describing teratomas care was particularly taken to emphasize the fact that many cases of duplicity of parts depended on dichotomy. Cleavage may be so slight at the cephalic end of the embryo as only to involve the face, or even the jaws. Of this I have described several specimens, RECTAL TERATOMAS 437 which make it clear that precisely the same thing takes place in connexion with the jaws as with the pelvic limbs. When this is the case, the supernumerary maxillae fuse together and are impacted in the naso-pharynx and fixed to the base of the sphenoid, or hang as a pedunculated tumour in the naso-pharynx. I have examined a large number of specimens (many of which are preserved in the splendid Teratological Collection of the museum of the Royal College of Surgeons), in which every gradation is traced, from well- formed maxillae with unerupted teeth to a confused lump consisting of teeth, bone, and cartilage impacted in the palate but firmly united by a broad base to the sphenoid in the neighbourhood of the pituitary fossa. Teratomas connected with the rectum and colon.— In order to appreciate the nature of such tumours arising in the immediate neighbourhood of the rectum, it will be necessary to consider a few points connected with the embryology of this portion of the alimentary, canal. In the early embryo, the central canal of the spinal cord and the alimentary canal are continuous around the caudal extremity of the notochord. This passage, which brings the developing cord and gut into such intimate union, is known as the neurenteric canal. When the proctodseum invaginates to form part of the cloacal chamber it meets the gut at a point some distance anterior to the sjDot where the neurenteric canal opens into it ; hence there is for a time a segment of intestine extending behind the anus, and termed in consequence the "postanal gut." Afterwards this postanal section of the embryonic in- testine disappears. There is good reason to regard the postanal gut as the source of that variety of congenital sacro-coccygeal tumour which was named by Braune and several writers who followed him " congenital cystic sarcoma." These will be referred to as tumours of the postanal gut. In addition, it will be necessary to consider dermoids situated between the rectum and the hollow of the sacrum — postrecta.l teratomas — and certain pedunculated tumours situated Avithin the rectum — rectal teratomas. Tumours which arise in the postanal gut exhibit a definite structure; they are composed of closed vesicles lined with glandular epithelium, and contain glue-like fluid. Many of 438 TERATOMAS these tumours are composed of cysts and duct-like passages lined with cubical epithelium, held together by richly cellular connective tissue. In many situations the epithelium is columnar, set upon flatter cells. The cysts are filled with ropy mucus, and vary in size from a nut to the smallest space visible to the naked eye ; many contain intracystic processes. These tumours present such definite characters that they are sure to attract attention, and their large size makes them very conspicuous. Middeldorpf was the first to associate clearly a congenital sacro-coccygeal tumour with the postanal gut. His specimen was removed from the neighbourhood of the anus of a girl a year old. The tumour contained connective tissue, mucous Fig. 219. — Rectal teratoma which contained brain-suhstance enclosed in a hony capsule : from a woman aged 25. {After DanzeJ.) membrane with characteristic follicles, submucous tissue, and longitudinal and circular laj^ers of muscle-fibres. I had come to the same conclusion in regard to the probable origin ot these tumours before the publication of Middeldorpf 's paper ; his case is the most conclusive on record. Postrectal teratomas are very rare, and do not form such large projecting masses as the preceding species. In many instances they are not noticed until after infant life, and their clinical tendencies are of a different character. An excellent example of a postrectal teratoma exists in the Middlesex POSTBEGTAL TERATOMAS 439 Hospital Museum : it contains grease, hair, and a tootli. It was found in the course of a post-mortem examination. The tumour remains in situ on the rectum. Such tumours also occur as surgical surprises, especially when they attain very large dimensions and extend upwards behind the pelvic peritoneum of men and women. Ord recorded a remarkable case which occurred in a man 28 years old ; the mass weighed fourteen pounds. Page successfully removed a teratoma, weighing three pounds, which occupied the hollow of the sacrum in a woman of 47 years ; it lay Fig. 220. — Cfficum and adjacent portion of the ileum of a man : a dermoid occupies tlie angle between the ileum and the cfficum. (The specimen is in the possession of Mr. A. Hall, ShefEeld.) behind the rectum. The pultaceous matter was evacuated through an incision in the perineum ; the cyst-wall was then successfully enucleated. Skutsch has recorded two examples of postrectal tera- tomas, and collected the chief German cases. One of the records states that the patient was pregnant, and he was able to empty and partially enucleate the tumour through an incision in the perineum without disturbing the pregnancy. Postrectal teratomas sometimes open spontaneously in the perineum ; the fistula is usually situated in the middle line of the perineum near the tip of the coccyx. Keen removed a 440 TERATOMAS postrectal tumour from a girl 3?, years of age; in the middle there was a fistula which led upwards to the third piece of the sacrum ; it contained fat, cartilage, etc. The tubular tract resembled a trachea, and possessed imperfect rings of cartilage, and was lined with ciliated epithelium. Teratomas of the rectum.— Several examples have been described grooving from the mucous membrane of the rectum (Fig. 219) ; a curious feature in these cases is that the tumours are furnished with long locks of hair, which pro- trude from the anus and annoy the patients (Danzel, Port). Like postrectal teratomas, they sometimes contain teeth. Nearly all the recorded examples of rectal teratomas have occurred in women, and this formerly gave some support to the suggestion that they arose in the ovary, and eroded their way into the rectum. In one recorded case a teratoma was found between the layers of the mesosigmoid; the patient died in consequence of an operation performed for its removal ; at the autopsy a dermoid was found in the connective tissue of the pelvis. The ovaries were normal (Moynihan). A pedunculated teratoma hanging from the mucous mem- brane of the sigmoid flexure led to intussusception in a girl aged 16 (Glutton). The study of dermoids and teratomas connected with the rectum is important and puzzling : some of them exhibit the characters of teratomas, and others should find a place with the simpler varieties of dermoids. The idea that some of them are included foetuses is reasonable when they are situated around the terminal section of the gut, but this can scarcely be entertained when the tumour, as in the case described by Moynihan, is in relation with the sigmoid flexure of the colon. A remarkable dermoid is represented in Fig. 220, lodged in the angle formed by the junction of the ileum and the csecum : the tumour lies between the layers of the peritoneal fold extending from the termination of the ileum to the mesentery of the vermiform appendix. It contained the usual pulta- ceous matter and hairs. The cavity was lined with stratified epithelium, but lacked a stratum granulosum. The sj^ecimen was obtained in the course of a post-mortem examination on the body of a man by Mr. Arthur HaU, who kindly gave me every facility for examining the specimen. REFERENCES 441 Bostroem, " Ueber die pialen Epidermoide, Dermoide, und Lipome, und duralen Dermoide." — Centrabl.f. path. Atiat., 1807, 1. Braune, W., " Die Doppelbildungen und angeboren Geschwulste der Kreuz- beingegend," 1862, 40 ct seq. Buzzard, F., Trans. Path. Soc, 1904, Iv. 330. Glutton, H. H., "Pedunculated Dermoid Tumour from the Sigmoid Flexure." —Trans. Path. Soc, 1886, xxxvii. 252. Middeldorpf, K., "Zur Kenntniss der angeboren Sacralgeschwulste."^ Vircliow's Arch. f. path. Anat., 1885, ci. 37. Moynihan, B. G. A.— Lancet, 1898, i. 80. Ord, W. M., and Sewell, C. B., " An account of a Large Dermoid Cyst found in the Abdomen of a Man." — Med.-Chir. Trans., 1880, Ixiii. 1. Page, Frederick, " Large Extraperitoneal Dermoid Cyst successfully removed through an Incision across the Perineum, midway between the Anus and Coccyx."— Brit. Med. Journ., 1891, i. 406. Port, Heinrich, " Dermoid Tumour from the Rectum." — Trans. Path, Soe. 1880, xxxi. 307. Rows, E. G,, " Two Cases of Embryoma in the Frontal Lobes of the Brain." —Rev. of Nexirol. a/nd Psyehiatry, 1906, iv. 338. Skutsch, F., " Ueber die dermoid Cysten des Beckenbindegewebes." — /£itschr. f. Gel. und Gyn., 1899, xl. 353. CHAPTER XLIV SEQUESTRATION DERMOIDS Dermoids are tumours furnished with skin occurring in situations where this structure is not found under normal conditions. They only possess the structures normal to skin, such as hair, sebaceous and sweat- glands ; teeth are rarely present. Dermoids may be arranged in two genera — 1. Sequestration dermoids. 2. Tubulo-dermoids. Sequestration dermoids arise in detached or sequestered portions of skin, chiefly in situations where, during em- bryonic life, coalescence takes place between cutaneous surfaces. A sequestration dermoid occasionally takes the form of a skin-lined recess, but more commonly it assumes the form of a globular tumour with a central cavity lined with skin, furnished with dermal elements. Dermoids of the trunk. — These occur strictly in the regions where the lateral halves of the body coalesce. This line of union, commencing immediately below the occipital protuberance, extends along the middle of the back to the coccyx ; it then passes through the perineum (scrotum and penis in the male") and upwards through the umbilicus, thorax, neck and chin, to terminate at the margin of the lower lip. Dermoids are rare along the dorsal part of this line and are apt to be mistaken for spina bifida sacs, especially when situated in the lumbo-sacral region. A man aged 22 had a congenital tumour in this region which had been regarded as a spina bifida sac (Fig. 221). It had never caused him inconvenience until a few days before his admission into the hospital, when it inflamed, burst, and discharged a quantity of foul-smelling sebaceous material mixed with 442 DEBMOinS OF TEE TRUNK 443 hairs. The cavity was freely opened and cleared of de- composing material. The skin lining the interior of the dermoid was beset with pores of large size, corresponding to the orifices of sAveat-glands ; when the patient perspired, drops of sweat could be seen oozing from these pores. This skin also contained nerves, for the man could localize the prick of a pin on the interior of the dermoid as easily as one made upon the skin surrounding the tumour. When the Fig. 221. — Dermoid in the lumbo- sacral region of a man 22 years of age. tumour was removed, the spinous processes underlying it were found to be unusually short and surrounded by fat. Rarely dermoids are associated with spina bifida. Gilbert Barling observed such a combination in a child 2 years old affected with spina bifida occulta ; the skin covering the de- fective spines presented the hair-field usual in these cases. In the tissues immediately over the stunted spinous pro- cesses a dermoid was found containing sebaceous material and hair (Fig. 222). It is very rare to find dermoids within the spinal canal. 444 DERMOIDS An interesting instance of this has been recorded by Hale White. It grew in the thoracic region of the spine, and produced paraplegia. Laminectomy was performed on the patient, a man 26 years of age, but the operation was not successful. Faulty coalescence of the cutaneous covering of the back often occurs over the lower sacral vertebrae, and gives rise to small congenital sinuses known as "postanal dimples" and " coccygeal sinuses." These recesses are lined with skin fur- nished with hairs, sebaceous and sweat- eflands. Sometimes they measure 10 mm. in depth. As a rule they are single, and often accompany lumbo-sacral spinal bifida. Though most commonly seen over the coccygeal or the last two Fig. 222. — Section of three thoracic vertebras with a small dermoid situated over two stunted spinous processes. sacral vertebrae, I have seen them as high as the fourth lumbar vertebra, and always exactly in the middle line. These, dimples are interesting, for — as will be shown after- wards — in many situations where sequestration dermoids occur, similar cutaneous recesses are also seen. An examina- tion of such a sinus serves to show that if its external orifice became occluded, without the deeper parts becoming obliter- ated, we should have the germ of a dermoid, for the numerous glands in the walls would be active, and their secretion, with the shed epithelial scales and hairs, would soon cause it to enlarge and assume such proportions as to render it recognizable as a tumour. The coccygeal sinuses are sometimes troublesome, as hair and dirt accumulate in them and lead to suppuration. Clini- SCROTAL DERMOIDS 445 cally a suppurating coccygeal sinus simulates an anal fistula, but a little care will prevent the surgeon from confusing between the two. A good physiological type of such a sinus is furnished by the interdigital pouch of the sheep. This pouch (Fig. 223) lies between the digits, and all the dissection required to expose it is to separate the digits with a sharp knife, keeping close to the phalanges of one or the other side. In adult sheep it is always full of shed wool and grit. Sometimes its orifice is occluded and it becomes a retention cyst ; Fig. 223. — Median aspect of a sheep's digit, showing the interdigital pouch. suppuration foUoAvs, much to" the sheep's discomfort. The Avails of this pouch are full of very large glands. In order to get satisfactory sections, it is necessary to obtain the digits from a still-born lamb, for as soon as lambs run about grit gets into the pouch and spoils the edge of the knife. Dermoids of the scrotum and labium. — There are many good reasons for believing that the majority of dermoids reported as arising in the testicle were really scrotal in origin. This was clearly the case in a specimen described by Bilton Pollard as a dermoid of the testicle. The dermoid was situated on the left side of the scrotum, betAveen the 446 DERMOIDS testicles, and adhered to the back of the left one outside the tunica vaginalis. It contained puttj^-like material in Avhich there were a few grey hairs. The cyst was lined with stratified epithelium ; papillse and sebaceous glands were detected. Dermoids have been described in relation with the inguinal canal. The only record which can be relied on is that by H. J. Fig. 224. — Dermoid situated over the junction of the manubrium and gladiolus of the sternum ; there was also a dermoid near the left cornu of the hyoid bone. The youth was 19 years of age. {After Bramann.) Paterson : he removed a cyst of this kind from the inguinal canal of a man 35 years of age. The microscopic examination in this case was very thorough. Dermoids of the labium are very rare : on one occasion I saw one removed as big as an orange from the right labium of a woman 40 years of age. It contained the usual pultaceous material and shed hair. The dermoid had burrowed beneath the deep fascia of the thigh and come into relation Avith the tendon of the adductor lonsfus muscle. THOU AG 10 DERMOIDS 447 Dermoids of the thorax. — Judging froin tlie few available records, dermoids of the thorax are very uncommon. They occur in two situations — viz. on the anterior aspect of the sternum and in the thoracic cavity. Dermoids on the front of the sternum are situated in the middle line near the junction of the manubrium with the gladiolus (Fig. 224) ; it is not uncommon to find a small cutaneous recess in this situation exactly in the middle line and resembling the .^' Fig. 225. — Dermoid in the epistemal notch ; it contained hair and pultaceous matter, and was superficial to the deep cervical fascia. coccygeal sinus. Sternal dermoids have been described by Bramann, Cahen, and Glutton. An unusual situation for a dermoid is the episternal notch (Fig. 225), and it is easy to understand that one in this situation could burrow into the superior mediastinum. At first o-lance it would seem difficult to account for the presence of a large dermoid within the thorax, but a review of the mode of development of the sternum throws much clear light on the subject. The two lateral halves of the sternum are, in the early embryo, widely separated from each other ; gradually they coalesce in the middle line. Every anatomist 448 DERMOIDS is aware that this median coalescence is extremely liable to be faulty, and conditions occur like those which, happening in connexion with the medullary folds, produce spina bifida. In this line of coalescence, so far as sternal dermoids are concerned, we may get skin-lined recesses resembling the coccygeal dimples. These sternal recesses, or dimples, occur near the junction of the manubrium with the gladiolus, and may be more than a centimetre deep. Should a piece of skin become sequestrated during coalescence of the thoracic walls, it may, during the development of the sternum, be dislocated forwards to the outer surface, or backwards towards Fronto-nasal plate. Globular process. Maxillary process. Mandibular process. Interuasal fissure. Orbito-nasal fissure. Mandibular iissure. lutermandibular fissure. Fig. 226. — Head of an early embryo to show the fronto-nasal plate, globular processes, and associated fissures. {Modified from His.) the mediastinum, conditions in every way parallel to the variations in the position of cranial dermoids. So long as a dermoid on a deep surface of the sternum remains small it will cause no trouble, but it is easy to understand that a large tumour would, if projecting into the thorax, encroach on the pleura. Even then it would not produce much disturbance so long as air did not gain access to it ; but if by pressure the wall of the cyst becomes so thin as to allow air to enter its cavity, or an actual communication forms between the cyst and a bronchus or the air-sacs of the lung, then suppuration with all its disastrous consequences will ensue. (Intrathoracic dermoids and teratomas are considered at p. 484). Facial dermoids. — Dermoids occur on the face in certain FACIAL DERMOIDS 449 definite positions, such as the inner and outer angles of the orbit ; on the upper eyehd ; in the naso-facial sulcus ; on the cheek slightly posterior to the angle of the mouth ; in the middle line of the chin, and on the nose. In order to appreciate the origin of dermoids in these situations it is necessary to bear in mind the relation of the facial fissures in the embryo, which in the adult are re- presented by the orbits, lachrymal ducts, mouth, and certain furrows in the lips and cheek. In the early embryo the face is represented by an opening Fig. 227. -Face with black lines to indicate the situation of the embryonic fissures. from which six fissures radiate (Fig. 226). The upper pair are the orbito-nasal ; the lower, the mandibular; the fifth and sixth are the internasal and intermandibular fissures. The median fold projecting into the opening from above is the fronto-nasal process, which ultimately forms the nose. As it develops, a rounded prominence, known as the globular process, forms at each angle and gives rise to a portion of the ala of the nostril and the corresponding premaxilla. These globular processes fuse together in the middle line to -form the central piece, or philtrum, of the upper lip. The elonga- tion of the fronto-nasal process necessarily lengthens the orbito-nasal fissures. Eventually the sides of the fronto- nasal plate coalesce superficially with the maxillary processes 2 D 450 DERMOIDS in such a way as to leave a cleft on each side, which becomes the orbit ; the line of union being permanently indicated in the adult by the naso -facial sulcus or groove, and indicated still more deeply by the lachr3^mal duct, which is a persistent portion of the original orbito-nasal fissure. The union of the fronto-nasal plate with the maxillary processes completes the nose, cheeks, and upper lip (Fig. 227). Fig. 228. — Eight side of the head of a fcetus, showing a large mandibular tubercle and an accessary tragus. The above account indicates in a general way the relation of these fissures to each other ; but it will be necessary, in considering dermoids arising in them, to mention certain details connected with each. But here it may be stated that the defects associated with any of them are of four kinds : 1, the fissure may persist ; 2, it may close imperfectly and leave a recess or puckering of the skin ; 3, portions of the surface epithelium may be sequestered and give rise to dermoids ; 4, there may be excessive coalescence. These conditions may be illustrated by the mandibular MANDIBULAR TUBEBGLE8 451 fissure. In the embryo this fissure or cleft is relatively more extensive than the opening of the mouth which in the adult ultimately represents it. In fishes the whole of the mandi- bular fissure persists as the gape ; but in mammals the dorsal portions of the clefts are obliterated by the union of their margins, leaving the central portion as the mouth. Persistence of the whole length of the fissure is a rare defect, and is known as macrostoma, while excessive closure of the fissure produces microstoma. Imperfect union of those ^ ^^^ ■! Fig. 229. — Head of a dog showing the mandibular tubercle. sections that normally coalesce gives rise to slighter imper- fections, of which some examples will now be described. Occasionally we find on one or both cheeks of children, at a spot varying from 2 to 4 cm. behind the angle of the mouth, a small nodule rarely exceeding a rape-seed in size. Some- times there is a depression or sinus in the cheek, surmounted by the nodule. Occasionally the buccal mucous membrane presents a shallow recess, sometimes a sinus, and occasionally a white cicatrix at a spot corresponding to the nodule on the cutaneous surface of the cheek. These mandibular tubercles and recesses are frequently 452 DERMOIDS associated with malformations of the corresponding auricles, as well as other facial defects, such as coloboma of the eyelid and pilose cutaneous patches on the conjunctiva. The largest specimen which has yet come under my observation occurred in a still-born foetus (Fig. 228). On the right cheek, 2 cm. behind the angle of the mouth, was a nodule the size of a rape-seed, and immediately behind this a pedunculated body. The tubercle on the cheek consisted of dense connective tissue traversed by blood-vessels and covered with skin beset with lanugo and richly supplied with sweat- and sebaceous glands of large size. In many mammals, especially dogs, small cutaneous nodules furnished with vibrissse may often be detected in a line with the angle of the mouth (Fig. 229). These nodules occupy positions corresponding with those of the mandibular tubercles of children. There is very little relationship between pathology and poetry, but that very philosophical pathologist, Sir Samuel Wilks, in reference to my observation that the usual position of the mandibular tubercle and recess corresponds with that of the dimple in the baby's cheek, drew my attention to the following passage in his Harveian Oration, 1879: "From any point of view we take, and upon whatever subject we fix our gaze, we come to the conclusion that the greatest discovery ever made by man about himself, and of the earth of which he forms a part, is the doctrine of evolution. " ' The softest dimple in a baby's smile Springs from the whole of past eternity, Tasked all the sum of things to bring it there.' " Wilks observed to me how little the poet (Miss Bevington) divined that there is a material basis for these three pretty and significant lines. Jevons expressed the same truth in the following epigram : " The origin of everything that exists is wrapped up in the past history of the universe." The intermandibular fissure. — When the mandibular pro- cesses fail to coalesce, the result will be a median cleft in the lower lip extending to or even beyond the chin. Median clefts of this kind are very rare. Occasionally such a defect is associated with a dermoid or a pair of small nodules in the skin. In terriers such nodules are almost constantl}'' present MANDIBULAR TUBERCLES 453 between the symphysis and the body of the hyoid bone. In children with double hare-lip two sinuses are sometimes seen in the mucous membrane of the lower lip. Their orifices are indicated by small but prominent papillse. The sinuses are large enough to admit a probe, and they are in some cases 2 cm. deep. Mucus exudes from them, furnished by glands which beset the membrane lining their walls. These sinuses Fig. 230. — Mother and her two children with mandibular recesses, hare-lip. {From a photograph.) Each had double are probably due to faulty coalescence of the intermandibular fissure. This view is strengthened by an observation of Feurer, who detected a similar sinus in the upper lip of a lad on the right side of the philtrum ; it corresponded exactly to the termination of the naso-facial fissure. For a remarkable observation in regard to mandibular recesses I am indebted to Mr. Nicoll. A mother and her two children had each a pair of recesses in the lower lip (Fig. 230). Each had double hare-lip, and the cicatrices of successful operation are clearly visible. The mother was one of a family of five, and each had double hare-lip and a pair of recesses in the lower lip. 454 DERMOIDS For a long time I thought that these recesses probably had a morphological significance, and made a Avide search through the various families of the mammalia for a type, but without success. A careful description of the histology of these sinuses is furnished by Madelung. The earliest recorded example in British literature is by Arbuthnot Lane, Bramann, F., " Ueber die Dermoide der ISlase." — ArcJi. ■^. Min. Chir. (Langenbeck), 1890, xl. 101. Cahen, Fritz, " Schweissdrilsen-Eetentionscyste derBrust." — Deutsche Zeitschr. f' Chir., 1891, xxxi. 370. Glutton, H. H., "Large Dermoid Cyst over the Sternum." — Trans. Path. Soc, 1887, xxxviii. 393. Feurer, G., "Angeborene Oberlippenfistel." — Arch.f. Jclin. Chir. (Langenbeck), 1893, xlvi. 35. Lane, W. Arbuthnot, " A Case in which two Sj'mmetrical Congenital Mucus- secreting Cavities existed in the Lower Lip." — Trans. Clin. Soc, 1891, xxiv. 230. Madelung, "Zwei seltene Missbildungen des Gesichts." — Arch.f. 1dm. Chir. (Langenbeck), 1888, xxxvii. 271. Paterson, H. J., " Dermoid Cyst of tlie Inguinal Canal." — Trans. Path. Soc, 1903, liv. 149. Pollard, Bilton, " Dermoid Cyst of Test\(i\Q."— Trans. Path. Soc, 1886, xxxvii. 3i2. White, W. Hale, " Dermoid Cyst attached to the Front of the Pericardium." — Trans. Path. Soc, 1890, xli. 283. White, W. Hale, " A Case in which the attempt was made to remove a Dermoid Tumour which, growing in the Spinal Canal, pressed upon the Spinal Covd."— Trans. Clin. Soc, 1900, xxxiii. 140. CHAPTER XLY SEQUESTRATION DERMOIDS (Concluded) Dermoids of the orbito -nasal fissure. — Dermoids appear in this fissure in three situations : (1) at the outer angle of the orbit ; (2) at the inner angle of the orbit ; (3) in the naso- facial sulcus. Of the three situations, by far the most frequent is the outer angle of the orbit, where they form rounded tumours rarely exceeding the dimensions of a cherry ; Fig. 231. — Dermoid at the outer angle of the orbit. they lie in close relation with the pericranium covering the frontal bone, which is often deeply hollowed to accommodate them. Dermoids in this region vary somewhat in regard to their position ; sometimes they are quite close to the external angular process of the frontal bone, or they may be 2 cm. or more posterior to it (Fig. 231) ; exceptionally they are on a level with, or even lie beneath, the eyebrow. 455 456 DERMOIDS Dermoids at the inner angle are far less frequent (Fig. 232). In this situation the tumour may extend beyond the bone and lie in intimate relation with the dura mater. It is very necessary to remember this in attempting the extirpation of the dermoid. In some cases the tumour may have a peduncle continuous with the dura mater. Under such conditions the Fig. 232. — Dermoid at the inner angle of the orbit. dermoid may transmit the cerebral pulsation ; it is then apt to be mistaken for a meningocele. Dermoids occur not only at the orbital angles, but some- times also in the tissue of the upper eyelid, unconnected with either bone or periosteum. These smaller dermoids probably arise in the fissure between the fronto-nasal plate and the cutaneous fold from which this eyelid is formed. The fissure between the two parts which form an eyelid sometimes persists. To this defect the term coloboma of the eyelid is applied. NASAL DERMOIDS 457 Dermoids arising in the orbital angles are the simplest of all dermoids, and though the skin lining them is usually rich in the ordinary cutaneous elements, such as hair, sebaceous and sweat- glands, complex structures such as teeth and bone, so far as my knowledge extends, have not been observed in them. I have satisfied myself that the skin in these dermoids is sensitive and that it possesses tactile sensibility. Dermoids in the lower section of the orbito-nasal fissure are rare. They usually protrude in the naso-facial sulcus, and occasionally possess a tooth (Fig. 233). Fig. 233. — Dermoid m the naso- facial sulcus containing a tooth. (After Paul) Fig. 234.— A translucent dermoid at the bridge of the nose. The man was 30 years of age. Nasal dermoids. — It is necessary to point out that in addition to the naso-facial sulcus, dermoids occur in two other situations on the nose. A not uncommon position is the bridge of the nose (Fig. 234). This part of the face is not traversed by a fissure, and the mode by which such a dermoid arises is in all respects identical with that which gives rise to cranial dermoids. In the skull of an early embryo, the fronto-nasal plate which ultimately forms the nose consists of a lamina of hyalin cartilage covered externally with skin and internally 458 BEBM0IB8 witli mucous membrane. After the third month, sections made through the nasal capsule, immediately anterior to the ethmoid, show that the skin is being dissociated from the underlying cartilage by bony tissue, which eventually becomes the nasal bones. Ultimately the cartilage disappears as a result of the pressure exercised by these bones. It is reason- able to believe that, in the gradual separation of the skin from the cartilage of the fronto-nasal plate by the intrusion of the nasal bones, small portions of skin or epithelium become sequestrated and eventually develop into dermoids. This explanation is more fully set forth in the next section, on dermoids of the scalp and dura mater. Fig. 23o.-Dernioid recess in the ^^S" 236.-Dennoid recess at the tip nose of an adult. °f ^^^ ^°^« °f ^ ''^^^'^- ^^he hair is represented as too coarse.) Dermoids near the tip of the nose are the consequence of faulty fusion of the internasal fissure, and usually take the form of narrow skin-lined recesses furnished with hair, which is often long enough to sprout beyond the recess (Figs. 235 and 286). Hair-lined recesses in the mid-line of the nose at some point between the lower border of the nasal bone and the tip of the nose are very common, but they rarely call for treat- ment. The}?- occur far more frequently in men than in women. In their mode of origin and characters they agree with the hair-lined sinuses known as postanal dimples. A much rarer anomaly than a dermoid is excessive CRANIAL DERMOIDS 45S coalescence of the nasal segment of the orbito-nasal fissure (Fig. 237). Dermoids of the scalp and dura mater. — The common situations for dermoids of the scalp are over the anterior fon- tanelle (bregma) and occipital protuberance. In these situa- tions they are occasionally confounded with sebaceous cysts or with meningoceles. Dermoids of the scalp often have a thin pedunculated attachment to the dura mater, the pedicle traversing a hole in the underlying bone, unless the cyst is over a fontanel le. The term " wen " used to be applied indifferently to se- baceous cysts and dermoids of the scalp. Sir Astley Cooper, Fig. 237. — Child with a deformed nose due to excessive coalescence of the nasal section of the orbito-nasal fissure. The case was under the care of Mr. Nicoll. in his essay on " Encysted Tumours," even included orbital dermoids among wens. In describing them, he writes : " The largest size I have known them acquire has been that of a common-sized coco-nut, and this grew upon the head of a man named Lake, who kept the house called the ' Six Bells ' at Dartford. It sprang from the vertex, and gave him a most grotesque appearance, for when his hat was put on, it was placed upon the tumour and scarcely reached his head. The cyst is in the collection at St. Thomas's Hospital, also a cast of his head taken just prior to the operation " (Fig. 238). 460 DERMOIDS The cyst, wiiich is probably the largest dermoid of the scalp on record, contains a number of round balls, some having a diameter of 1 cm. These consist of epithelial cells mixed with fat. (See also Sibthorpe and Marsh.) Arnott published the details of an instructive case of a dermoid situated over the anterior fontanelle in an infant a Fig. 238. — Head of the man Lake witli a large dermoid over the bregma. {From a cast in the Museum, St. Thomases Hospital.) few days old. The tumour exactly resembled a meningocele, " rising and falling with regular pulsation, and swelling when the child coughed " ; the resemblance was so strong that it was regarded as a meningocele. A few weeks later the child died from broncho-pneumonia, and the cyst was found to be a dermoid. The specimen is preserved at St. Thomas's Hospital. (See also Giraldes.) Dermoids in the neighbourhood of the occipital protuber- ance (inion) may lie on the inner aspect of the occipital bone, and are nearly always in relation with the tentorium CRANIAL DERMOIDS 461 cerebelli. Examples have been described by Turner, Ogle, Pearson, Irvine, and Lannelongue. They occurred in children, and in Ogle's case there was defective development of the squamous portion of the occipital bone. In Lannelongue's patient, a girl 7 years old, the dermoid had attained the size of an orange; it produced marked symptoms, such as paralysis, amaurosis and coma, ending in death. Although at first sight a dermoid connected with the dura mater and projecting into the brain seems to -violate all embryological rules, nevertheless, when we view this membrane from a morphological standpoint the strangeness vanishes and a satisfactory explanation is forthcoming. Morphologically considered, the bony framework of the skull is an additional element to the primitive cranium, which is represented by the dura mater, and, as I have else- where endeavoured -to show, the term extracranial should strictly apply to all tissues outside the dura mater. In sur- gical practice we find it convenient to regard the bones as the boundary of the skull, but morphologically this is in- accurate ; the skull-bones are secondary cranial elements. Early in embryonic life the dura mater and skin are in con- tact; gradually the base and portions of the side- walls of the membranous cranium chondrify, thus separating the skin from the dura mater. In the vault of the skull, bone develops between the dura mater and its cutaneous cap, but the skin and dura mater remain in contact along the various sutures even for a year or more after birth. This relation of the dura mater and skin persists longest in the region of the anterior fontanelle (bregma) and the neighbourhood of the inion. Should the skin be imperfectly separated, or a portion remain persistently adherent to the dura mater, it would act precisely as a tumour-germ and give rise to a dermoid. Such a tumour may retain its original attach- ment to the dura mater, and its pedicle become surrounded by bone; the dermoid would lie outside the bone, but be lodged in a depression on its surface, with an aperture trans- mitting its pedicle. On the other hand, the tumour may become separated from the skin by bone ; it would then pro- ject on the inner surface, or between the layers of the dura mater. If this view of the origin of dermoids of the scalp be 462 DERMOIDS admitted, we must then modify our teacliing, and say that the depressions in which dermoids of the cranium are lodged arise as imperfections in the developmental process, and are not due to absorption induced by pressure ; further, the fibrous connexion of such dermoids with the underlying dura mater is primary, not accidental. A study of the development of the tentorium cerebelli will demonstrate that it is composed of two folds of dura mater, and it arises as an infolding or crease in this membrane, caused by the rapid backward extension of the developing cerebrum. The opposed surfaces of the tentorial lamellae, like the outer surface of the dura mater in relation with the cerebrum, were originally in contact with the skin, and as the posterior margins of the bay or recess formed by the crease in the dura mater come together, a portion of the skin may become nipped or even sequestrated between the layers of the tentorium ; this, preserving its vitality, and in some cases its cutaneous connexions, may ultimately give rise to an intracranial dermoid. IMPLANTATION-CYSTS These small cysts should not be included among tumours, but their consideration is imperative in connexion with sequestration dermoids, for they furnish valuable evidence that dermoids of this genus arise from " rests," the result of faulty coalescence. These cysts are caused by the accidental implantation of portions of skin, epithelium, or hair-bulbs in the underlying connective tissues. The transplanted tissue acts in many instances as a graft, and forms a small cyst. Implantation- cysts have received a variety of names, such as dermal cysts, traumatic dermoids, sebaceous cysts of the fingers, etc. (Fig. 239). They are common on the fingers, the cornea, and the iris, but may arise on any part of the skin. They have been ob- served by many surgeons, and careful accounts have been written, especially by Polaillon, Le Fort, and Garre. Implantation-cysts vary much in size ; some are scarcely as big as a split pea, others may be as large as a ripe cherry. In many the microscopic characters " a]3pear as if a piece of IMPLANTATION- CYSTS 463 the skin covering the pulp of the finger had been inverted " (Shattock). In others the implanted epidermis seems to have been shed in layers, so that on section the interior of the cyst is occupied by epithelial laminae. When these cysts occur on the scalp, the interior contains hair. Implantation- cysts are caused in a variety of ways, such as punctures by awls, forks, needles, thorns, glass, etc. ; also accidental wounds by knives, incisions by scalpels, bites, and lacerations. These cases are of interest, for they serve to throw light on some cysts, containing hair and wool, preserved in the museum TV""-''''-^ .- Fig. 239. — Implantation-cyst of the finger. of the Royal College of Surgeons. Two of the cysts are from sheep, and contain wool embedded in fatty matter. Unfor- tunately, the catalogue affords no information as to the region of the body whence they were removed. The third and fourth specimens were removed from the shoulder of a cow that had six legs. The cysts contain light hair, fatty and calcareous matter. These four specimens are Hunterian. The iifth specimen was removed from beneath the integu- ments of the shoulder of an ox. It contained slender black hairs, resembling those on the skin of the animal, mixed with fat. I once obtained a good example of an implantation-cyst from the axilla of an ox. The cyst was as large as a billiard- ball, and in structure resembled a piece of inverted skin. Fortunately, these cysts can be explained on the same lines as similar cysts of the fingers in man. The sticks used by cattle-drovers are armed at the end with a sharp iron spike, 2-5 cm. (1") long, with which they "prod" the beasts, often very severely. It may be assumed that punctures produced with such an instrument may lead to the deposition of dermal grafts beneath the skin, which may give rise to cysts 464 DERMOIDS in the same way as punctured wounds in the sldn of men and women. Punctured wounds in sheep and oxen may also be caused by projecting nails, iron spikes, tenter-hooks, and the hke. The opinion that cysts may arise in the subcutaneous tissues by implantation receives the strongest jDossible con- firmation from what we know of similar cysts of the Ms and cornea associated with mechanical injury. Iritic cysts. — Cysts of the iris are of comparative rarity, generally appearing as transparent vesicles situated on its anterior surface. As a rule they are sessile, but occasion- ally possess a pedicle. The contents may be opaque, but in exceptional cases they have been filled Avith sebaceous material, such as fills the cavities of dermoids. Hulke (1869) collected some valuable facts in relation to such cysts, and states that in fifteen out of nineteen cases, as well as in two reported by himself, there was distinct history of antecedent mechanical injury. Numerous instances are known in Avhich eyelashes, some- times as many as six, have been implanted on the iris by foreign bodies penetrating the cornea, such as knives, needles, foils, and swords. Barry Sullivan, whilst acting as Richard III., received during the famous combat (Act v., Scene 4), a wound in the eye from his opponent's sword. Subsequently a cyst containing an eyelash grew from the iris. Similar cysts have been produced in the eyes of rabbits by the artificial introduc- tion of eyelashes and epithelium into the anterior chamber. Corneal cysts. — In addition to the evidence furnished by implantation-cysts of the iris, we know that similar cysts occur in the cornea. Treacher Collins has investigated this matter, and has published some valuable researches in which he has succeeded in demonstrating that after gunshot injuries of the eyeball, blows from tip-cats, and incisions made for the extraction of cataracts, cysts, usually of small size, are liable to form in the cornea near the seat of injury. In some of the specimens the cysts may be very large and conspicuous ; when examined microscopically, their inner walls are found lined with layers of ceUs identical with those covering the anterior surface of the conjunctiva. The structure of these cysts, taken in conjunction with the antecedent injuries. SEQUESTRATION DERMOIDS 465 tlioroughly supports the view that they arise from conjunc- tival epithehum transplanted into the deep tissues of the cornea, Arnott, Henry, " Dermoid Cyst o£ the Scalp simulating Meningocele." — Trans. Path. Soa., 1874, xxv. 228. Ashby, H., and Wright, G. A., " Diseases of Children, Medical and Surgical," 1899, p. 770. [Dermoid containing tooth.] Bland-Subton, J., "A Critical Study in Cranial Morphology." — Juurn. of Anat. and Phys., 1888, xxii. 28. Collins, E. Treacher, "The Anatomy and Pathology of the Eye," 1896,. pp. 77, 78. le Fort, " Kyste du Petit Doigt. Eecidive apres une premiere ponction ; guerison par la compression. Analyse chimique du liquide." — Bull, et 3Imi. de la Soc. Chir., 1881, vii. 547. Garre, C, " Ueber traumatische Epithelcyste der Finger." — Beit. z. Min. Cliir. (Bruns), 1894, xi. 524. Giraldes, J., " Le9ons Oliniques sur les Maladies Chirurgicales des Enfants," 1869, 342. Hosch, Fr., " Experimentelle Studien liber Iriscysten." — Virchow's Arch, f -path. Anat., 1885, xcix. 449. Hulke, J. W., " On Cases of Cysts of the Iris." — Roy. Bond. Ophthal. Hosp. Repts., 1869, vi. 12. Irvine, J. Pearson, " Dermoid Cyst of the Brain." — Trans. Path. Soc, 1879, XXX. 195. Lannelongue et Menard, V., "Kystes Extra-crauiens de I'lnion." — -"Affections Congenitales," 1891, i. 50, 51. Marsh, F., " Dermoid Cyst simulating a Meningocele." — Brit. Med. Journ., 1900, i. 443. Ogle, J. W., "Congenital Cysts containing hair and sebaceous material, or communicating with the cranial sinuses [Morbid growths of the brain, spinal cord, etc.] " — Brit, and For. Med.- Chir. Bev., 1865, xxxvi. 208. Paul, F. T.," Dermoid Tumour of the Face, carrying Teeth." — Trans. Path. Soc, 1894, xlv. 148. Polaillon, " Doigt (Pathologic) ; Kystes Dermoides." — Die. Ency. des Sci. Med., 1884 (lere serie), xxx. 281. Sibthorpe, " Congenital Sebaceous Cyst." — Brit. Med. Journ., 1888, i. 350. Turner, William, " Case of Intracranial Cyst containing Hair ; also a Case illustrating the physiological action of iodine." — St. Bart's Uoi-i). Bepts., 1866, ii. 62. Walther, C, " Kyste de I'lnion."— Prme Mkl., 1895, pp. 123-126. 2 E CHAPTER XLVI TUBULO - DERMOIDS LINGUAL DERMOIDS-MEDIAN CERVICAL FISTUL^E— ACCESSARY THYROID GLANDS There exist in the human embryo certain canals and passages many of which normally disappear before birth. Among these obsolete canals there are three that require especial consideration in connexion with dermoids — viz. the thyro- glossal duct, the postanal gut, and the branchial clefts. The remainder will be considered in the section devoted to cysts. The thyro-glossal duct. — The thyroid gland of man consists of two lobes united by a narrower portion or isth- mus. His maintains that the three parts of this gland arise separately. The lateral lobes originate independently ot the isthmus, which is derived from a median tubular out- growth from the ventral wall of the embryonic pharynx, known as the thyro-glossal duct. This duct bifurcates at its lower end and gives rise to the thyroid isthmus, which fuses with the lateral thyroid rudiments, and assists in forming the lobes of the gland. Originally the duct extends as far upwards (forwards in the embryo) as the dorsum of the tongue, but as the body of the hyoid bone develops, the duct becomes divided into an upper segment, the lingual duct, and a lower portion, the thyroid duct. In the ordinary course of develop- ment these ducts disappear, but in some cases they persist and attain a fair size. Thus the central part of the thjroid may be regarded as the remnant of a secreting gland provided with a duct Avhich conveyed the products of the gland into the pharynx. There are at least three abnormalities which appear to be associated with vagaries of the thyro-glossal duct: (1) lingual dermoids, (2) median cervical fistulse, (3) accessary thyroids. 466 TUSULO-BERMOIDS 467 1. Lingual dermoids. — Dermoids arising in the tongue have been many times observed and reported as sebaceous cysts. Barker, however, pubhshed a clear account of their nature, and showed them to be true dermoids. Subsequent research has proved that those dermoids which occupy a central jDosition in the tongue between the genio-hyo-giossi muscles arise in the lingual duct. When fully developed this duct extends from the foramen csecum to the posterior surface of the basi-hyoid. Occasionally the duct is so large that a Fig. 240. — Large lingual dermoid, protruding from the mouth. {Gray.) probe may be introduced into it from the foramen csecum. The duct lies exactly between the genio-hyo-glossi muscles, and is not infrequently replaced by a solid fibrous cord. It is easy to understand that if a persistent duct should have its upper end obstructed or obliterated, the continual shedding of the epithelium which lines it and the accumulation of sebum from the glands would convert it into a cyst, which in due course would assume such a size as to come within the range of clinical observation. The walls of lingual dermoids are composed of fibrous tissue, lined internally with squamous epithelium beset with 468 DERMOIDS hair and sometimes with glands. The contents of these cysts are epithehal cells, hair, sebum, and cholesterin. Should the cyst burst, then it would suppurate and become very disagreeable. Lingual dermoids are occasionally sufficiently large to attract attention in infants, but most of the examples come under notice in adolescents (Fig. 240). In addition to the common variety of dermoid, the tongue is occasionally occupied by tumours which in structure re- Fig. 241.— Median cei-vical fistula in a man aged 23 years. The fistula appeared when he was 3 years old. semble the thyroid gland. They occur in the neighbourhood of the foramen caecum, between the genio-hyo-glossi muscles. Bernays has given a careful description of such a tumour which he removed from the tongue of a girl 17 years of age, and associated the tumour with the lingual duct {also Wolff, Warren, and Mcllraith). 2. Median cervical fistulae. — These openings occur singly, and open at some point in the middle line of the neck between the hyoid bone and the top of the sternum. The common MEDIAN GEBVIGAL FISTULA 469 situation is a little below the level of tlie cricoid cartilage. Median cervical fistulse differ from those arising in connexion with branchial clefts in the fact that they are never congenital; they may occur soon after birth or make their appearance as- late as the fourteenth year, Cusset described a median cervical fistula in 1877, but LE\f,h/. FECIT. Fig. 242. — Section of a persistent thyroid duct. A represents the duct of natural size. The lowest drawing shows the epithelium more highly magnified. Raymond Johnson clearly pointed out that median cervical fistulse are preceded by a swelling in the middle line of the neck which either ruptures or is opened by the surgeon ; this leaves a sinus which never closes. The following is a common example of a median cervical fistula. The patient presented in the lower third of the neck 470 DERMOIDS a depression, the floor of wliicli was puckered and scar-like (Fig. 241). At the upper part of this bay or recess there was a rounded opening from which clear mucus exuded. An ordinary probe introduced into this hole easily passed upwards in the middle line directly beneath the skin, to stop at the middle of the lower border of the basi-hyal. The opening in the neck had existed as long as he could remember, but his parents told him that it appeared when he was about Fig. 243. — Median cervical fistula associated with a persistent thyroid duct. three years old. Ordinarily the fistula caused no incon- venience, but during the past two years it seemed subject to catarrh, and the excessive flow of mucus caused him much inconvenience, so it was dissected out. The duct was lined with columnar ciliated epithelium. The tissue forming its walls resembled atrophied th3^roid tissue ; here and there (Fig. 242) isolated channels could be seen in section lined with columnar epithelium. MEDIAN GERVIGAL FISTULA 471 Occasionally a persistent thyroid duct is so large as to form a conspicuous vertical ridge in the middle of the neck in association with a median cervical fistula (Fig. 243). Thus a median cervical fistula is in striking contrast to branchial fistulse, which are always lateral in position and in close relation with the anterior border of the sterno-mastoid inuscle, and are always congenital. Our knowledge of the nature of these fistulas was not very satisfactory until the publication of an able paper by Marshall, detailing the anatomy of the parts in the neighbourhood of the hyoid bone of a child 5 years old, who had a median Foramen caecum. Hyoid boae. Thyroid cartilage. Pyramid of thyroid gland. Abscess sac. Tliyroid gland Trachea. Fig. 241. — Diagram to show the relation of parts in a case of median cervical fistula. {After C. F. Marshall.) sinus in the neck. The patient was admitted into a hospital for the purpose of having the duct excised; it contracted diphtheria and died before the operation could be performed. In the median line of the neck, 2-5 cm. (1") above the sternum, there was a sinus, which, during life, discharged a small quantity of mucous fluid. From this opening a hard cord could be felt extending up to the hyoid bone. On dissecting the front of the neck this cord was found to be 472 DERMOIDS tubular and patent up to within 1 era. of its termination ; the upper end was firmly attached to the hyoid bone, the lower end dilated into a thin-walled sac opening on to the surface of the skin. The sac and tube lay between the skin and the anterior layer of the deep cervical fascia ; at no place was there any connexion with the thyroid gland. On dividing the hyoid bone the tube could be traced as an ill-defined fibrous cord on to its dorsal surface, to which it was closely attached, and through the substance of the tongue to the foramen caecum. About 2 cm. from the foramen it again became patent, and continued so up to the surface of the tongue. The canal was thus open at both ends, but impervious in the middle. On further dissection a lobus pyramidalis was found connected with the left side of the thyroid isthmus, its upper end being united to the median fibrous cord at the same place as the above-mentioned canal In other words, the fibrous cord behind the hyoid bone was continuous both with the pyramidal lobe of the thyroid and with the tube leading to the superficial sinus (Fig. 244). The relations of the parts indicate the probable mode by which these median fistula arise ; they are probably retention- cysts formed in a persistent thyroid duct, and the pressure of the cyst ultimately causes the skin to yield and form a sinus. 3. Accessary thyroids. — The consideration of accessary thyroids naturally follows on the description of median cervical fistulse, for there is good reason to believe that the thyroid duct is the source of some of these bodies. They have long been known (Albers and Virchow), and in recent years have been carefully studied. They occur most frequently in the neighbourhood of the hyoid bone and in the hollow formed by the two lobes of the thyroid gland. As the thyro- glossal duct is directly associated with the formation of the thyroid body, and as median accessary thyroids are found directly in its track from the hyoid to the thyroid isthmus, it is not unreasonable to regard these little bodies as remnants of this remarkable tube. Accessary thyroids occasionally arise in connexion with the germs of the lateral lobes of the thyroid : these are most ACCESS ART THYROIDS 473 commonly found in the neighbourhood of the greater cornu of the hyoid. Accessary thyroids are in the main innocent structures, but occasionally they give rise to troublesome tumours. It is well known that when the thyroid body becomes goitrous, and accessary thyroids co-exist, the latter will enlarge and become, in fact, goitrous. Apart from this, accessary thyroids will enlarge on their own account and produce tumours which closely simulate unilateral enlargement of the thyroid, and occasionally give rise to bronchoceles of moderate dimensions. Albers, " Atlas der pathologischen Auatomie," 1847, Abth. ii., Taf. xxv., xxvi. and xxix. Barker, A. E., " Sebaceous or Dermoid Cyst of the Tongue ; removal by sub- mental incision ; cure." — Trans. Clin. Soc, 1883, xvi. 215. Barker, A. E., " Two Cases of Dermoid Cyst in connection with the Tongue." — Trans. Clin. Soc, 1891, xxiv. 68 (p. 70, " Case of Dr. Wellington Gray "). Bernays, A. C. — St. Louis Med. and Surg. Journ., Iv. 201. Johnson, Raymond, " Two Cases of Persistent Thyroid Dact." — Trans. Path. Soc, 1890, sli. 325. Mcllraith, C. H., " Notes on a Case of Accessary Thyroid Gland projecting into the Mouth."— i^rJ!^. Med. Jonrn., 1894, ii. 1234. Marshall, C. F., " The Thyro-Glossal Duct or ' Canal of His.' " — Journ. Anat. and Phys., 1892, xxvi. 94. Warren, J. Collins, " A Case of Enlarged Accessary Thyroid Gland at the Base of the Tongue." — Amer. Journ. Med. Sci., 1892, civ. 377. Wolf, R., " Ein Fall von accessorischer Schilddruse." — Arch. f. klin. Chir. (Langenbeck), 1889, xxxix. 224. CHAPTER XLVII CERVICAL FISTULA, DERMOIDS AND AURICLES CERVICAL FISTULiE AND AURICLES Cervical fistulae. — It is not imcommon to find in the neck, at some point along the anterior border of the sterno- mastoid muscle, a small orifice in the skin capable of admitting a bristle or a fine probe. These congenital openings are known as cervical or branchial fistula3, and they are probably persistent representatives of the branchial fissures which were dis- covered in the embryos of pigs, horses, and man by Rathke in 1825 (Fig. 245). Congenital fistulous openings in the side Fig 245 —Early ^^ ^^^ nock wcro obscrved many years be- human embryo, forc Rathke's embryologic discovery, and shoM-iug the gill- Heusinger (1854) was the first clearly to recognize the relationship of these fistulse with the branchial clefts. In the majority of cases these openings terminate as sinuses, but exceptionally they pass deeply among the struc- tures of the neck and terminate on the wall of the pharynx or open into the j)haryngeal cavity. One, two, or three orifices may be present in the same child, and they exhibit a great tendency to be bilateral, to affect several members of the same family, and to be transmitted to several generations. These sinuses or canals, which may vary in length from 2 to 5 cm., are lined by mucous membrane, sometimes with ciliated epithelium, or by skin containing sebaceous glands. The lining membrane of the canal usually secretes a thin mucous fluid, which may become increased during catarrhal conditions of the respiratory passages. Occasionally the canal inflames and an abscess results, which ma}^ give rise to considerable pain and difficulty in deglutition. The external orifice of a branchial fistula may 474 GEBVIOAL FISTULJ^ 475 be indicated by a tag of skin containing a piece of 3'ellow elastic cartilage, and these protuberances are commonly known as cervical auricles. The external orifices of the sinuses vary in j)osition, but they are always situated along the anterior border of the sterno-mastoid muscle. The common situation is a spot in line with the angle of the jaw, but they may open anywhere along the line of the muscle from the mastoid process to the sterno- clavicular articulation. When the fistula extends to the pharynx, the duct keeps a constant course and passes between the fork of the carotid artery, above the sling of the superior laryngeal nerve, and terminates in the sacculus pyriformis. Heuter refers to a young man who had a cervical fistula and " wished to become a trumpeter " ; he dissected out the fistulous tract, " following it between the two carotids to the pharynx." A lad of 15 years under my observation complained of a mucous discharge which soiled his collar occasionally ; fluid when swallowed leaked through. I dissected out the duct and found that it passed through the fork of the carotid artery. Heusinger held the opinion that some pharyngeal diver- ticula arise as distensions of the persistent pharyngeal seg- ments of branchial clefts. Morrison Watson recorded a case in which he made a careful dissection of such a diverticulum. The parts are shown in Fig. 246, and in the description it is stated that a tube terminating inferiorly in a cul-de-sac containing a large quantity of grumous material was found extending from the pharynx, immediately behind the tonsils, to the interclavicular notch. This tube possessed muscular walls, and in the deep part of its course passed between the fork of the carotids and over the loop of the superior laryngeal nerve ; its lower part was parallel with the anterior border of the sterno-mastoid muscle ; it rested on the sterno-hyoid and sterno-thyroid muscles. It communicated with the pharynx by means of a slit-like opening, not more than 3 mm. in length, the margins of which were so closely in contact that the entry of solid particles into it from the mouth must have been prevented. The diverticulum itself increased in calibre from above downwards, so that whilst at the upper end a 476 DERMOIDS crow-quill could 'witli difficult}^ be introduced, at the lower a pencil could readily be passed along the lumen of the tube. It is further noteworthy that the pharyngeal orifice was situated between the lower jaw and the stylo-hyoid ligament. Its point of departure from the pharynx corresponds to the supratonsillar fossa. The muscle-fibres were, for the most part, red and striated, and the mucous lining resembled that of the ffisophagus. It has long been suspected that the so-called sebaceous Fig. 246. — Pharyngeal diverticulum. {After Morrison Watson.) cj'sts which arise in the neck below the deep fascia take origin in unobhterated segments of branchial clefts. Such a C3^st does not necessarily contain grease or hair : it may be filled with mucus. The walls of cervical fistulEe are covered with epithelium of various kinds, which in some is ciliated and in others squamous, and so forth. Mucous cysts in the side of the neck arising in persistent branchial clefts must not be confused with lyrnphatic cysts (see p. 164), or with der- moids associated with the thjTo-glossal duct {see p. 466). Rowley has described and figured a small tumour which GEBVIGAL AURIGLE8 477 he found in a frog, Rana temporaria, posteriorly to the angle of the jaw. This on microscopic examination was found to be made of concentric laminae of epidermis and dermis. The structure and position of the tumour led Rowley to regard it as a dermoid due to the inclusion of epithelium during the occlusion of a gill- cleft in larval life. Cervical auricles. — In describing branchial fistulse (p. 475) it was mentioned that the cutaneous orifices are in some cases surmounted by tags of skin. These tags, or processes, sometimes occur unassociated with fistulse, but Fig. 247.— Cervical auricles in a child. always in situations where fistulse, when present, open on the skin. Usually they are short, in some cases mere nodules, but in others form prominences 2 to 3 cm. in height. These processes have been described under a variety of names, and classed among tumours, but at the present time they are commonly known as cervical auricles. Like branchial fistulas, they are always congenital, and sometimes affect several members of a family. The mother may have a cervical auricle, and one of her children a branchial fistula, whilst another child may have an auricle associated with a fistula ; they are often symmetrical (Fig. 247). 478 DERMOIDS A cervical auricle consists of an axis of yellow elastic carti- lage, which sometimes extends deeply into the tissues of the neck; muscle-fibres from the platysma are attached to the cartilage, and the whole is surmounted with skin con- taining hairs and sebaceous glands. A small arterial twig runs into the auricle and ramifies in the fibrous tissue and fat in which the cartilage is embedded. Thus, structurally, cervical auricles are identical with the 1^ V ■A" ^% Fig. 248. — Head of a goat with cervical auricles. normal auricle or pinna, and they agree with the pinna mor- phologically, inasmuch as they are developed like it from that portion of a branchial bar which is directly in relation with the corresponding cleft. In sharks the gill-shts open separately on the surface of the body ; from the branchial bar anterior to each slit a fold of skin is formed, which closes upon the sht like a lid and is named from this resemblance the operculum. In mammalian embryos a slight prominence or tubercle is for a time visible anteriorly to each of these clefts. In most cases GERVIGAL AURICLES 479 the tubercles disappear from the posterior bars, but those in relation with the anterior cleft enlarge and are joined by accessary tubercles to form the pinna. Thus embryology has taught me to regard the pinna as consisting mainly of an operculum which has become modified for acoustic pur- poses, for we may regard the tubercles formed in relation with the branchial clefts of man as representative of the opercula of certain Ichthyopsida. As the pinna is mainly derived from opercular tubercles, and cervical tubercles, in all probability, represent persistent opercular tubercles, it is reasonable to term them cervical auricles. Fig. 249. — Head of a homed sheep with cervical auricles. The homology of at least a part of the pinna and cervical auricles with the opercula of fish has been made clearer by Schwalbe's discovery of auricular tubercles in the embryo of the turtle (Emys lutaria taurica) ; in the adult condition chelonians have no vestige of an auricle. Cervical auricles occur in mammals other than man. Heusinger, in 1876, mentioned the frequency with which pendulous tags of skin occur in the necks of pigs, goats (Fig. 248), and sheep (Fig. 249) ; yet very little has been done to extend his observations. The anatomy of these auricles (which are especially common in Egyptian and Italian goats') is similar to that 480 DERMOIDS of cervical auricles in man : there is an axis of yellow elastic cartUage embedded in fibrous tissue and fat, the whole being covered with hairy skin. In Great Britain cervical auricles are rare in pigs, but Pro- fessor Anderson Stuart has drawn attention to the existence in Australia of a breed of pigs known as the Bell-pig on account of the presence of pendulous folds of skin in the neck (Fig. 250). It may here be mentioned that in Germany these auricles in sheep and pigs are known as Glockchen or Berlocken. ^N\ Fig. 250. — Head of a pig with cervical amides (the Bell-pig of Australia). Before concluding the subject of cervical auricles, reference must be made to the presence of these appendages on the necks of satyrs. Mr. Shattock drew my attention to the fact that in the statues of many satyrs we find in the neck, in the situation where cervical auricles are usuall}^ found, promin- ences which in their variety of form resemble the cervical auricles of goats and men. In the eegipans (goat-footed satyrs) the auricles in the neck are pointed like their ears and are sessile, but in the fauns they are usually pendulous (Fig. 251). In the statues of many satyrs, both fauns and segipans, no auricles are represented, and they are less con- AUBIGULAR FISTULJE 481 stant in modern than in ancient statues of fauns, and in some they are unilateral. The hircine element is particu- larly evident in the segipans, even in their tails (Fig. 346). AURICULAR FISTULA AND DERMOIDS We may assume that the auricle or pinna consists mainly of an enormously developed operculum which ha,s become Fig. 251.— faun and goat with cervical auricles. {From the Ccqntol.) utilized for acoustic purposes. It has already been pointed out that in the embryo each branchial cleft is surmounted by a swelling or tubercle corresponding to the operculum of the shark. In mammals, and, as Schwalbe has shown, in reptiles, the first cleft, which ultimately becomes modified into the tympano-Eustachian passage, is surrounded by additional tubercles, some of which belong to the mandibular and others to the hyoid bar (Fig. 252). It is by the subsequent growth 2 F 482 DERMOIDS and coalescence of these tubercles that the auricle is formed. These tubercles have received from His the following names : I., tuberculum tragicum ; ii., tuberculum anterius ; iii., tuber- culum intermedium; iv., tuberculum anthelicis; v., tuberculum antitragicum ; and vi., lobulus. The subsequent fate of these tubercles may be briefly given. The tuberculum tragicum unites across the cleft with the tuberculum antitragicum, the space formerly separating them being simply indicated by the incisura intertragica. The tuberculum intermedium is the source of the helix, whilst the tuberculum anthelicis furnishes the anthelix : the nodule vi., cut off by the fusion of tragus and anti tragus, becomes the lobule. Imperfections in the development and union of these Fig. 252. — Two di-awings representing the development of the auricle {sec text ahove). [Modified from Sis.) tubercles will serve to explain several congenital defects to which the auricle is liable. Of these, three are of especial interest: (1) auricular fistulee; (2) auricular dermoids; (3) accessary tragus. 1. Auricular fistulse. — Heusinger seems to have been the first to describe a congenital fistula in the helix. For the first complete account of these fistulse in England we are indebted to Sir James Paget. The fistula usually appears as a small opening. leading into a canal ending blindly in the substance of the helix. The auricle may be of good shape, but often it is deformed (Fig. 253). Usually a small quantity of greasy material exudes from the orifice of the sinus, ivhich varies from 2 to 6 mm. in depth. These fistulse sometimes exist AURICULAR FISTULA 483 in individuals who also have branchial fistuke ; or one mem- ber of a family will have a congenital fistula in the auricle, and another a congenital fistula in the neck : they are hereditary. It is far rarer to find congenital fistulse in the lobule. Very few examples have been observed. A little girl known to me was born with a perforation in the lobule of the left auricle exactly in the spot for wearing an earring, and to this day she wears a ring in this lobule and refuses to have the other pierced. Fig. 2.53.— Congenital fistula in the helix. {After Paget.) The facts now at our disposal enable us to understand how such fistulse arise, for it seems reasonable to conclude that if the various lobules which conspire to form an auricle unite imperfectly, the intervening spaces will persist as sinuses or fistuke. 2. Auricular dermoids.— From what has just been stated' regarding the probable mode of origin of auricular fistulse, it will be obvious that if unobliterated skin-lined spaces are left between the tubercles uniting to form the auricle, and the skin lining such spaces possesses glands (sequestered tracts of 484 DERMOIDS skin are unusually rich in sebaceous glands), we have in such spaces potential dermoids. The auricle is not an uncommon situation for cysts often described as sebaceous ; they are usually small, but sometimes attain the dimensions of a cherry, or even larger. When these supposed sebaceous cysts are examined microscopically they sometimes turn out to be dermoids. It is a curious fact that unless small dermoids in unusual situations are very carefully examined, they run a great chance of being put aside as sebaceous cysts. Auricular dermoids of fair size sometimes occuj)y the groove between the pinna and the mastoid process ; if allowed to grow they will form a deep hollow in the imderlying bone. Fig. 254. — Auricle with a duplicated tragus. Fig. 255. — Aui-icle of a fcetus with an unusually large Woolner's tip f\rmished with a tuft of lanugo. 3. Accessary tragus. — One of the commonest malform- ations of the pinna is duplication of the tragus. The accessary tragus is extremely variable in shape ; often it assumes the form of a low conical projection in front of or above the tragus (Fig. 254) ; sometimes it is pedunculated and hangs as a small cutaneous tag slightly in front of the tragus, beset with pale, delicate hair. It is curious that an accessary tragus, a Woolner's tip (Fig. 255), and a mandibular tubercle (Fig. 228) are usually covered with long lanugo. Occasionally an accessary tragus is associated with a circular cicatris-like depression in the cheek immediately in front of the pinna. It is a fact of some interest that malformations of the tragus and an accessary tragus are often associated with defects in the mandibular fissure, such as macrostoma, mandibular fistula, and tubercle. AURICULAR TUBERCLE 485 Woolner's tip. — This name lias been given to a small tubercle often present on the margin of the helix (Fig. 255). It was noticed by Woolner, the celebrated sculptor, whilst he was at work on his statuette of Puck, to whom he gave pointed ears. The urchin is " perched upon a toadstool and with liis toe rousinoc a froof." Woolner drew Darwin's atten- tion to this tubercle whilst modelling a bust of the famous naturalist. After a careful consideration of the facts, Darwin thought it probable " that the points are vestiges of the tips of formerly erected and pointed ears." Woolner made his observation at the age of 22. I possess a fine example of this famous statuette. Heusinger, " Hals-Kiemen Fisteln von noch nicht beobachteter Form." — Arch./. path. Anat. (Virchow), 1864, xxix. 358. Heuter, C, " Grundriss der Chirurgie," 1882, ii. 328. His, W., "Anatomie menschlicher Embryonen," 1885, Heft iii. (Die For- mentwickelung des ausseren Ohres), p. 211. von Kostanecki, K., " Beitiage zur Kenntniss der Mi.ssbildungen in der Kopf nnd Halsgegend." — Arch. f. path. Anat. (Virchow), 1891, cxxiii. 401. Paget, Sir J., " Cases of Branchial Fistulas in the External Ears." — Med.-Chir. Tram., 1878, Ixi. 41. Rowley, " Tumour found in a Frog posterior to the Angle of the Jaw." — Trans. Leicester Lit. and Fhilosoph. Soc, April, 189G. Schwalbe, " Ueber Auricularhocker bei Eeptilien ; ein Beitrag zur Phylogenie des ausseren Ohres." — Anat. Anzeiger, 1891, vi. 43. Watson, Morrison, " Notes of a Remarkable Case of Pharyngeal Diverticulum." — Journ. Anat. and Phys., 1874-5, ix. 134. CHAPTER XLVIII TUMOURS OF THE FEMALE GENITAL GLAND (OVARY) The ovary is a complex organ histologically and morphologi- cally : it is with extraordinary frequency the source of tumours, some of them being so complex in character as to set at naught the ordinary rules of oncological classification. The frequency and clinical importance of ovarian tumours justify their consideration as a subdivision in a general description of tumours. The ovary consists morphologically of three parts : (1) the oophoron ; (2) the parooj)horon ; (3) the parovarium. Fig. 256. —Diagram representing the morpliologic regions of the ovary. A, Oiiphoron ; b, paroophoron ; c, parovarium (epoophoron) ; K, Kobelt's tubes ; G, Gartner's duct. 1. The oophoron. — This forms the free surface of the ovary, and may be described as the egg-bearing segment, for it contains the ovarian follicles. 2. The paroophoron. — This part forms the hilum of the ovary : it consists of fibrous tissue and blood-vessels ; it never contains ovarian follicles. In young ovaries glandular tissue 4SG TUMOURS OF THE OVARY 487 may be detected, remnants of tlie mesonephros (Wolffian body) from which it is mainly derived. 3. The parovarium (epoophoron). — A structure consist- ing of a series of tubules situated between the layers of the mesosalpinx. These tubules at their ovarian extremities ter- minate in the paroophoron ; at the opposite end they open into the duct of Gartner; this duct occasionally may be traced downwards to the vagina. The parovarium and the duct of Gartner are the persistent excretory ducts of the mesone- Fig. 257. — Cyst of the ouf)horon. {Xat. size.) A, Incipient cyst ; b, paroophoron ; F, Fallopian tube ; p, parovarium. phros ; in the female they are vestigial, but in the male they are functional as the excretory ducts of the testis. The tumours which arise in the ovary Avill be described in the following order, viz. : Dermoids or Embryomas ; Lutein Cysts ; Papillomatous Cysts ; Parovarian Cysts ; Gartnerian Cysts ; Fibroids ; Sarcomas ; Carcinomas. DERMOIDS (EMBRYOMAS) The ociphoron is the source of three varieties of tumour, known as cysts, adenomas (multilocular cysts), and dermoids (or embryomas), which in their type-forms are easily dis- tinguished, but they approach each other b}^ such gradations 488 TUMOURS OF THE OVARY as to make it difficult to draw a dividing line ; moreover, conglomerate tumours are occasionally found in the ovary, consisting of dermoids, cysts, and adenomas. Simple cysts. — These may be unilocular or multilocular. A small oof)horonic cyst is an enlarged ovarian follicle, and its walls are furnished with a well-developed membrana granu- losa. In a very early stage it is easy to demonstrate the relation of such a cyst to the oophoron. As the cyst enlarges it causes rapid absorption of the paroophoron, and the region in which it arose is then not so easily demonstrable. It is only by patiently waiting for opportunities of securing cysts in very early stages that it is possible to elucidate their mode of origin. Much of the confusion which obscures the pathology of this question is due to the fact that most in- vestigators have devoted their attention mainly to large cysts. In cysts containmg three or four litres of fluid the walls will be found to consist of fibrous tissue, and epithelium is rarely detected. It is impossible to state definitely the size of a cyst in which the epithelium disappears. The absence of epithelium is due to atrophic changes, the consequence of the contmual pressure exerted by the accumulating fluid. Precisely similar changes mscy be studied in the mucous membrane of greatly distended gall-bladders. In large multi- locular cysts, although the big loculi may be destitute of epithelium, the smaller cavities will retain their epithelium, which may be columnar, cubical, or stratified. An extremely simple means of determining an oophoronic tumour is to note the relation of the Fallopian tube ; it lies curled up on the cyst, and when the parts are stretched the tube and tumour are separated by the mesosalpinx. Adenomas (multilocular glandular cysts). — This is an important variety of tumour. It has a dense fibrous capsule, and its surface' is usually lobulated. These tumours attain colossal dimensions and consist of innumerable loculi and cysts which vary in size from a cavity no bigger than a pea to one holding a litre or more of fluid. Critical dissections of such cysts enable us to recognize three varieties of loculi. In typical specimens a honeycomb-hke mass will be found projecting into some of the larger cavities and occupying OVARIAN ADENOMAS 489 usually one-tliird of its circumference, so that a section of the cavity resembles a signet-ring. Such are called primary, whilst the cavities occupying the honeycomb portion are secondary cysts, and are, as a nratter of fact, mucous retention -cysts. The third set of loculi are of small size, and Fig. 258. — Portion of a large ovarian adenoma, showing the varieties of loculi. c, Primary ; d, secondary. histologically are indistinguishable from distended ovarian follicles (Fig. 258). The primary cysts in their early stages are lined with rich columnar epithelium, and often contain mucous glands (Fig. 259). The fluid contained in the loculi of ovarian adenomas is identical in its physical and chemical characters with mucus. Occasionally it is as thick and tenacious as jelly. The lining 490 TUMOURS OF THE OVARY of the cavities is in many specimens indistinguishable from mucous membrane. In some specimens of ovarian adenomas the secondary locuH give rise to projections on the periphery of the tumour ; when numerous and close together, these projections cause the tumour to resemble a colossal bunch of grapes. It is no uncommon thing for a loculus of an ovarian adenoma to burst into the belly. When this happens the mucus which escapes Fio;. 259. -Section of the wall of a loculus from an ovarian adenoma, showing the glandular disi^osition of the epithelium. is tolerated, but not absorbed, by the peritoneum. When the rent in the loculus is not repaired the glands in its walls continue to secrete, and the mucus accumulates in the belly, simulating hydroperitoneum. On one occasion I removed from a woman's belly three gallons of inspissated mucus of this kind which had been secreted by an ovarian aden- oma no bigger than a coco-nut. The belly was so tightly stuffed with this jelly-like material that it had produced a hernial protrusion at the umbilicus and the left femoral ring, the sac in each case beinof crammed with thick mucus. OVARIAN GYSTS 491 Ovarian cysts with ciliated epithelium. — As a rule the epithelium found in the type-forms of ovarian adenomas is tall and columnar, and it- may be ciliated (Fig. 261). In 1905 I removed a fist-sized unilateral ovarian tumour from a woman 45 years of age, and its semi-solid condition raised in my mind a suspicion of malignancy. On the instant of removal small pieces of the tumour were placed in preparation fluid and at once forwarded to Dr. Bashford. He Fig. 260.— Ovarian adenoma presentinga cutaneous clump (d) with a tuft of hair (/;). {Museum, St. Thomas's Hospital.) reported that the tumour contained ciliated epithelium, and that it was innocent in character. I subsequently examined the tumour after it had been carefully hardened, and the manner in which the solid parts are connected with the periphery of the tumour is exceptional (Fig. 261). The solid parts are made up of spaces lined with columnar cells ; in many of the cystic spaces the epithelium presents cilia. The view that the tumour is of an innocent nature seems correct, for a year later the patient was in excellent health. The source of this ciliated epithelium is a matter for conjecture, but Walthard, who published the results of a 492 TUMOURS OF TEE OVARY painstaking investigation into the histology of the human ovary at various ages, mentions the occasional existence of small cysts in this organ lined with C3'lindrical cells without any tendency to form warts, and in some the epithelium is columnar and ciliated. He also describes small epithelial bodies in the ovary which have no connexion with ovarian follicles, and which, he believes, arise from the cell-bundles Fig. 261. — Ovarian adenoma in section. The gland-spaces were lined with columnar epithelium, and some of them were ciliated. that dip into the ovarian stroma, and out of which the true ovarian follicles evolve. Dermoids (embryomas). — A very large proportion of oophoronic cysts contain skm or mucous membrane, or both these structures, and some of the many organs peculiar to them, such as hair ; sebaceous, mucous, and sweat- glands ; dermal bone, horn, nail, nipples and mammse ; teeth also occur in great numbers : such are called dermoids. They may be multilocular or unilocular, and attain a weight of 20 or even 40 kilogrammes. Sometimes a cvst will be lined OVARIAN DERMOIDS 493 throughout with typical mucous membrane covered with regular columnar epithelium, and will contain mucous glands. Fig. 262.— Ovarian embryoma. The lower part of the tumoui- contained teeth- germs in early stages of development, h, Tuft of hair. It is impossible to determine in many cases, from a mere naked- eye examination, whether an ouphoronic tumour should be regarded as an adenoma or a dermoid. In practice 494 TUMOURS OF THE OVABY the presence of a tuft of hair or a tooth is a useful and. ready- way of settling the question. Failing this, a careful micro- scopical examination is necessary. For instance, the tumour represented (nearly natural size) in Fig. 262 consists of two parts; one a thin- walled cyst (filled with sebaceous material when fresh) lined with piliferous skin. The lower and larger portion resembled, on superficial examiDation, an adenoma, and was nearly solid. A small tuft of lanugo-like hair in- duced me to make a careful histologic examination of the adjacent tissue. The sections revealed an extraordinary diversity of tissues and organs, such as sebaceous and sweat- glands, hair-germs, skin, teeth-germs with typical enamel- organs and dentine papillae, epithelial pearls, and shapeless masses of epithelium. Cysts occur in the oophoron at all periods of life, even in very young children, and I have collected records of over one hundred cases in girls under 15 years of age in which ovariotomy was a necessity from the size of the tumour. In one remarkable case an ovarian tumour from a girl of 15 years weighed 44 kilos; the girl weighed 27 kilos (Keen). Small cysts in the oophoron are very common at birth, and are often bilateral; but, so far as I am aware, after a careful and prolonged investigation of the matter, no authentic example of an ovarian dermoid has been observed in a child before the end of the first year of life. Adenomas and dermoids are very apt to affect both ovaries simultaneously ; very rarely two independent derm- oids may arise in one ovary ; and it is a fact that both ovaries may be so distorted and destroyed by dermoids that the true ovarian tissue is unrecognizable to the naked eye, yet such organs are able not only to dominate menstruation but to discharge their egg -bearing functions successfully (see Cullingworth, Thornton, and F. Page). Bilateral ovarian dermoids have been observed in a woman of 92 ; she was the mother of six children (Pollock). Rate of growth of ovarian adenomas and dermoids. — Concerning the rate of growth of these tumours very little is known. Therefore the following observations may be of interest : — 1. Ovarian adenoma. — In May, 1901, I removed from a OVARIAN DERMOIDS 495 woman 45 years of age a typical left ovarian adenoma ot the size of a football ; it was full of the usual colloid stuff. The right ovary was very carefully examined and found to be normal. In February, 1903, I removed from the same patient an ovarian adenoma of the size of a football, which had originated in the right ovary. Thus a complex glandular tumour of the size of a football may grow from an ovary apparently normal in twenty- one months. 2. Ovarian dermoid. — The following case is recorded by Fig. 263. — Enormous ovarian cyst in a girl 17 years of age ; it contained 78 litres of fluid. {After Bcnjot.) Flaischlen : In May, 1887, Ruge ovariotomized a woman, removing a cyst, as large as a child's head, which had arisen in the left ovary. The right ovary was inspected and found to be natural. In June, 1888, a tumour the size of a fist was detected on the right side of the pelvis. In December, 1890, laparotomy'- was again performed, and a dermoid, containing hair and teeth, removed. In this case the evidence is decisive that a dermoid 'may arise in the ovary and attain dangerous proportions in an ad.ult woman tuithin the space of three years. That a tumour containing hair and erupted teeth should be produced in the course of three years is not inconsistent with the rate at which these organs are formed under normal conditions. For instance, the period between the fertilization 498 TUMOURS OF THE OVARY of an ovum and the eruption of the milk incisors in man is about fifteen months ; in exceptional instances children are born with incisors above the gum. In such cases the process occupies less than nine months. The cutaneous organs found in ovarian dermoids pre- sent such extraordinary variation as to demand separate consideration. Skin. — This, in some specimens, is very thick, but it rarely possesses papillae, and when present they are not furnished with touch-corpuscles. Pigment is occasionally present. An epidermis may be demonstrated ; sometimes it is very thick, and the superficial layers are shed into the cavity of the dermoid in broad flakes. The usual arrangement of the epithelium resembles that which is found on the buccal mucous membrane rather than on skin in general, and there is no stratum granulosum. The subcutaneous tissue of dermoids is often particularly rich in delicate fat. In a few instances the epidermis has been found trans- formed into nail. An ill-formed nail has been detected, at the extremity of a piece of bone resembling a phalanx, by several observers. Hair. — This varies in length, colour, and amount. A single tuft coiled into a ball and mixed with sebaceous matter is not infrequent, and may attain a length of 50 cm. Munde has described and figured a specimen in which a tuft of hair in an ovarian dermoid was 1-5 metres long. Frequently only a few hairs are found scattered on the cyst-wall, or the hair may be rolled into balls and lie free in the cyst. Occasionally the shed hair will " felt." The colour is equally capricious, and, as a rule, differs from that on the exterior of the individual. The hair in such cysts changes in colour with age, and in elderly persons becomes quite white and is eventually shed, so that these cysts become actually bald. In an ovarian dermoid from a mare the hair resembled that on the animal's mane or tail (Pollock). In a similar tumour from a ewe, wool existed. Sebaceous glands. — The extraordinary abundance and large size of these glands are a conspicuous feature of typical ovarian dermoids. They are occasionally transformed into cysts, and, exceptionally, horns sprout from them. OVARIAN DERMOIDS 497 Sweat-glands. — These are not nearly so common as the sebaceous variety, and usually occur in irregular isolated clusters. 1 have as yet failed to detect the characteristic twist of the duct so constant in normal sweat-glands. Pidtaceoiis material. — The cavities of ovarian dermoids are often filled with a semi-fluid mixture of epithelium, sebum, fat, shed hairs, and often cholesterin. In small cysts rig. 26i. — Ovarian dermoid containing 3,930 epithelial balls. the sebum is sometimes so pure as to be quite white in colour. The fat may be liquid at the temperature of the body, but solidities after its removal. In some dermoids it occurs in lumps of the density and colour of cacao-butter. This variation probably depends on the proportion of stearin in the fat. Epithelial halls. — In some rare cases the shed epidermis forms rounded pill-like bodies which I ventured to call 2 G 498 TUMOURS OF THE OVARY epithelial balls. As a rule, three, four, or even twenty, and perhaps fifty, may be present. In one remarkable specimen which I examined, the number amounted to 3,930. Each contained one or more hairs as a nucleus, around which the epithelial masses cohered to form balls (Fig. 264). Bonney has reported a similar case, collected the literature, and attempted to demonstrate by experiment the probable mode by which these balls are formed. These pill-like bodies have been found in dermoids of the scalp and neck. Ovarian mamvue and teats. — It is quite common to find Fig 265. — Ovariau mamma with hair and teeth. in the interior of ovarian dermoids one or more tags of skin resembling a nipple or teat associated with hair and teeth (Fig. 265). These teats may be small and inconspicuous, but usually they are obvious to the most casual observer. Often they are attached to round, skin-covered prominences resembling mammse. These teat-like processes are imperforate and beset with large sebaceous glands. In some specimens the mamma is plump and well-formed, but consists entirely of fat covered with skin. The nipple may be surrounded with an areola. Complete forms are sometimes found with glandular acini ducts and a perforated nipple, and furnish a viscid fluid which exhibits the microscopic characters of milk and contains colostrum globules. OVABTAR DERMOIDS 499 The most complete ovarian mamma from a histological point of view is one described by Yelits: the nipple was surrounded by a rosy areola with clusters of Montgomery's tubercles, and small tufts of blond hair. Its structure was characteristic of a mamma (Fig. 266). Thyroid gland. — In 1893 I removed a large ovarian tumour from a woman 50 years of age, and detected in it a firm, rounded, encapsuled body as big as a walnut. Its cut surface so resembled a thyroid gland that I examined it microscopically. The body was composed of closed vesicles filled with colloid material and lined with the sub-columnar Fig. 266. — Histologic characters of the ovarian mamma described by Velits. a, Pigmented connective tissue ; b, plain muscle fibre ; c, d and c^ gland-acini and ducts. epithelium so characteristic of the normal thyroid gland. Similar observations have been recorded since by Kroemer, Bell, and Cleland. Bone. — This tissue is often present in ovarian dermoids in shapeless masses resembling the alveoli of jaws, or as irregular plates of extremely dense bone, " similar to the facial bones of an osseous fish " (Doran). Occasionally the fibrous capsule of a dermoid becomes calcified. Nerve-tissue. — Many observers have detected the presence of nerve-tissue in ovarian cysts. Gray (1858) described such a cyst which contained tissue indistinguishable from " cerebral 500 TVMOURS OF THE OVARY matter." In one instance tlie brain substance was enclosed in a firm capsule in structure like dura mater, lined with a delicate pia mater (Neumann). The museum of St. Thomas's Hospital contains an ovarian cyst in which nervous matter is present in the form of a tubular hydromyelic sac. This has been investigated by Shattock. The interior of the sac is lined with columnar epithelium, and the halves of a spinal cord lie on each side of it. The opposite ovary of the patient contained a hydromyelic sac, furnished with a delicate separ- able lining representing the meninges of the dilated nervous system, and an outer membranous wall. The nervous tissue consists of a reticulum of fine fibrils provided with cells having oval nuclei, and in it there lie many branching nerve-cells furnished with conspicuous nuclei and nucleoli. Cleland has described an ovarian dermoid containing cerebral substance dilated into a sac resembling a hj^clro- cephalus. " At one spot was a large mass of melanin granules surrounded by fibrous tissue." It is suggested that this may represent the choroid coat of an eye. This is a justifi- able interpretation. Bauingarten detected a curious eye-like organ in an ovarian dermoid. It presented a transparent portion shaped like a watch glass, and of the size of a pfennig, corresponding to the cornea. When bisected, it contained a cavity, the size of a cherry-stone, filled with clear fluid. The walls of the cavity were lined with a delicate layer of pigmented epithelium resembling the uvea of a normal eye. Nothing resembling a retina was detected. Pigmented tissue of the same kind, associated with neuroglia and neuro-epithelium, has been described in detail by Frank. The nervous tissue found m ovarian embrj^omas is very remarkable in another Avay : these tumours occasionally display malignant characters and disseminate in the abdomen {see p. 506). When these disseminated nodules are examined, many contain embryonic brain-substance such as neuroglia and ganglion cells (Backhaus). Backhaus, " Ueber ein metastasirendes Teratoma Ovarii." — Arch. f. Gyn., I'JOl, Ixiii. 159. Baumgarten, " Ueber eine dermoid Cyste des Ovarium mit angenahnlicben Bildangen." — Virchow's Arch., Bd. cvii. 515. OVARIAN DERMOIDS 501 Bell, R. Hamilton, " On the Appearance of Thyroid-like Structures in Ovarian Cysts. "—Trans. Obsfet. Soc, 1905, slvii. 242. Bland-Sutton, J., '■ An unusual example of Rupture of an Ovarian Adenoma." —Trans. Obstet. Soc. Lond., 1899-1900, xli. 98. Bonney, V., "A Dermoid Cjst containing a large number of Epithelial BnWs:'— Trans. Obstet. Soc, 1902-1903, xliv. 351. Cleland, J. B., " Two interesting Human ' Dermoid Cysts ' (Embryomata) : (1) containing Tliyroid Gland; (2) Cerebral 'Substance.'" — Austral. Med. Gaz., 1910, p. 235. CuUingwortli, C. J., " Three Cases of Suppurating Dermoid Cyst, of or near the Ovary, treated by Abdominal Section." — St. Tlwmas's Hasp. Bepts., 1887- 1889, xvii. 139. Frank, R. T., " A Case of Malignant Teratoma of the Ovary." — Amer. Journ. of Obstet., 1907, Iv. 348. Gray, H., " An account of a Dissection of an Ovarian Cyst which contained Brain." — Med.-CMr. Trans., 1853, xxxvi. 434. Kroemer, P., " Ueber die Histogenese der Dermoidkystome und Teratome des Eierstocks."— .4rc7<. f. Gyn., 1899, Ivii. 322. Munde, P. P., " A Switch of Hair five feet long from a Dermoid Cyst." — Trans. Kem YotJ: Obstet. Soc, 1891, Amer. Journ. of Obstet., xxiv. 854. Neumann, E., '• Doppelseitiges multiloculares Dermoidcystom mit Neubildung centraler Nervensubstanz (zwei seltene FJiUe von Ovarialcysten)." — Virchow's Arch.f.jmtli. Anat., 1886, civ. 492. Page, P., " Acute Peritonitis after Confinement ; abdominal section ; Dermoid Disease of both Ovaries ; removal ; recovery." — Lancet, 1893, ii. 250. Pollock, C. Stewart, "Cyst of the Ovary of a Mare (with report on the specimen)." — Trans. Obstet. Soc. Lond., 1889-1890, xxxi. 234, 253. Shattock, S. G., "Ovarian Teratomata." — Lancet, 1908, i. 479. Thornton, J. Knowsley, " A Case of Removal of both Ovaries during Preg- nancy."— Traws. Obstet. Soc Lond., 1886-1887, xxviii. 41. von Velits, D., " Eine Mamma in einer Ovarialgeschwulst." — Virchow's Arch, f. imth. Anat., 1887, cvii. 505. CHAPTER XLIX TUMOURS OF THE OVARY (Continoed) MATURE OF THE OVARIAN DERMOID (EMBRYOMA) It has been held by several writers during the past fifteen years, myself among them, that dermoids of the ovary differ in so man}?- respects from those found in connexion with the embryonic fissures (sequestration dermoids) that they require separate consideration from the taxonomic, anatomic, and genetic points of view. The idea that they arise from included pieces of epiblast I have always endeavoured to com- bat. Apart from other considerations, it must be remembered that sequestration dermoids are congenital, whereas there is no authentic observation of a dermoid existing in the ovary at birth : they occur in infancy and early girlhood, and often of large size. For some years I made a careful study of the ovaries of still-born foetuses, and instituted unremitting in- quiries amongst men who have specially interested themselves in the question, yet no specimen of this condition is available. This at once establishes a distinction between the sequestra- tion dermoid, the teratoma, and the so-called dermoid of the ovary. The diS'erence may be expressed in this way : — 1. A teratoma arises in embryonic life. 2. Sequestration dermoids are formed during foetal life. 3. Ovarian dermoids or embryomas are of postnatal origin. In its simplest form an ovarian embryoma is indistin- guishable from the common dermoid of the facial fissures. It is a cyst lined with epithelium furnished with hair. In a more complex form, in addition to hair it possesses teeth, bone, and secreting glands. In its highest form there are organs such as a piece of intestine, soft bud-like processes 502 EMBRY0MA8 503 composed ot brain-tissue, a well-formed vulva, a condition of things resembling an acardiac foetus (see p. 428). It has been pointed out by Wilms that an ovarian dermoid presents two parts, namely, a cyst and an embryonal rudi- ment. The cyst is composed of fibrous tissue arranged in wavy bundles : its inner aspect is lined with loose connective tissue, and at one part it presents a skin -covered surface of variable extent usually beset with hair. Associated with the skin-covered surface there is an " embryonal rudiment," Fig. 267. — Ovarian dermoid or embryoma containing a pseudc -mamma, (Museum, Eoyal College of Stirgeons.) usually in the form of a nipple-like process (pseudo-mamma) (Fig. 267). The size of this rudiment varies greatly; it may be so inconspicuous as to be easily overlooked, or so large as to strike the eye of the least observant; or the embryonal rudiment may approach the complexity of an acardiac foetus. Experience teaches that ovarian dermoids do not always conform to this simple plan of construction : specimens some- times come to hand containing many cysts, and each cyst contains a " rudiment " (Fig. 268) ; moreover, it is not un- common to find more than one nipple or pseudo-mamma in a cyst. The tissue underlying the skin-clad surface contains 504 TUMOURS OF THE OVABY glandular tissue, which occasionally is so abundant as to obscure the small cutaneous element. OVARIAN ADENOMAS Much careful attention has been given to the histology Fig. 268.— Ovarian dermoid (embryoma) comiDOsed of three cysts, each containing an "embryonal rudiment." r.t., Fallopian tube ; Jimb., tubal fimbrije ; t, teeth. of the complex ovarian embryomas, and many investigators have come to the conclusion that they arise from the im- perfect development of ova. This theory, however, does not satisfactorily explain the occurrence of an embryoma with teeth in the Fallopian tube (Orthmann) or in the testicle. EMBBY0MA8 505 In view of the opinion that the ovarian embryoma may be an attempt to form a foetus without impregnation, it is worth notice that in 1799 BaiUie, in describing an ovarian dermoid containing pilose skin, suet, and teeth, observes that this change has been generally considered as the very imperfect rudiments of a foetus which has been formed in the ovarium. As, however, the change takes place in the ovarium before the uterus would appear capable of functions which would begin at the age of puberty, and where the hymen is entire, it is highly probable that it is independent of impregnation. Interesting as all these questions are, the practical surgeon has to face the important clinical fact that some of these ovarian embryomas display malignancy in its most dangerous form, namely, the power of dissemination. This will now be considered. The malignancy of ovarian embryomas. — If we restrict the term dermoid to those ovarian tumour^ which contain typical dermic elements such as skin, hair, teeth, skin-glands, and the like, it may be truly said that they are the most benign tumours which attack women. There is, however, an interest- ing phenomenon connected with them requiring considera- tion, and which I have ventured to term epithelial infection. The details of several carefully described cases are available in which the peritoneum has been found dotted with minute knots, furnished with small tufts of hair, growing among visceral adhesions, even as high as the liver. (Moore, 1866, Kolaczek, Fraenkel, Grawitz, Lucy, Lawrence, and Melchior.) In each of these patients there was a dermoid in the ovary, and in the clinical reports of some of them there Avas a dis- tinct history of an injury to the abdomen, which makes it obvious that this condition could be explained by the epi- thelial contents of the dermoid escaping into the belly and becoming engrafted on the peritoneum. In Lucy's remark- able case the abdomen contained eleven pounds of pultaceous matter which had leaked from an ovarian dermoid. Before operation the abdomen pitted on palpation. The patient, a woman aged 32, noticed this herself. She recovered from the operation. If we widen the group of ovarian dermoids so as to em- brace the ovarian adenoma, which I maintain is pathologically 506 TUMOURS OF TEE OVABY correct, tlien we must include a rare variety of peritoneal infection unmistakably malignant. The most typical examples of ovarian adenomas may infect the peritoneum, I removed from a woman 51 years of age an adenoma the size of a football. Two years later she again came under my care, and I removed an ovarian adenoma of the opposite ovary which had burst and filled the belly with the usual gelatinous or colloid stuff. Six years after the second operation she came into my hands again with an enormously distended belly : at the operation the abdomen was found filled with colloid jelly, and the whole of the peritoneal surface covered with a nmltitude of small bodies which on microscopic examination exhibited the large columnar cells so characteristic of the ovarian adenoma. The patient was alive and well three years afterwards. In contrast to this the following facts are gloomy. I have records of three cases in which a tumour to the naked eye and to the microscope seemed to be an ordinary benign adenoma, but it had burst before removal and filled the belly with the usual viscous matter. Before these patients recovered sufficient strength to leave their beds, signs of re- current growth made themselves obvious, and some twelve weeks later the patients died with secondary deposits on the peritoneum. Recently cases of this kind have been reported under the name of malignant embryomas ; in some of them the peritoneal nodules contained cartilage, epithelial pearls, and ganglion-cells. It has also been shown that in some of the cases the secondary nodules assumed the form of grafts, and were in most instances confined to the peritoneum, but undoubted cases are known in which the malignancy assumed the form of visceral metastasis. In 1871, Jessop of Leeds recorded briefly a case illustrat- ing metastasis in connexion with a dermoid. The patient was a girl aged 13 years, and the tumour consisted of one large cyst with several smaller ones attached, and of a mass of white cheesy matter mixed with numerous thin, colourless, curly hairs. At the autopsy cancerous deposits were found in the liver, right suprarenal capsule, and mesen- teric glands. Malignant embryomas of the ovary are rare, and occur in LUTEIN GY8T8 507 young adults and in early childhood. Dudgeon reports a fatal example in a girl aged 3^- years. (For a careful report of a case and a summary of the literature, see Targett and Hicks.) The bearing of the evidence at present available indicates that the more closely the elements of an ovarian tumour conform to the adult type of tissue the more benign will be its clinical course. The more widely the glandular elements depart from the normal type the more prone will these elements be to infect the peritoneum, as the result of accident, or if in the course of an operation they be spread about the peritoneal cavity. My pathological inquiries convinced me of the import- ance of this observation, and since 1890 I have ceased to tap ovarian cysts in the course of removal, no matter what their size, but have removed them entire. LUTEIN CYSTS Perhaps the most familiar naked-eye feature displayed on the cut surface of a mature ovary is the yellow body known as the corpus luteum. All who have been seriously inter- ested in the pathology of ovarian cysts have noticed the frequency with which the corpora lutea are converted into cysts. Rokitansky drew attention to this, and held the opinion that they might enlarge and form tumours large enough to become clinically important. Cysts arising in corpora lutea do attain a size sufficient to admit of de- tection in the course of a careful bimanual examination. When these cysts are small their nature is easily determined by the thick layer of yellow material which lines them ; but as the cyst increases in size the lutein tissue is spread out and becomes less obvious, until it fades away and leaves a transparent thin-walled cyst which would not be regarded as a lutein cyst unless examined with the assistance of a microscope. It has been shown by Lockyer that an ovary may contain two or even a cluster of lutein cysts, and the condition may be bilateral ; in this event the consequent en- largement of the ovaries is such that on physical examination a tumour of some size can be detected on each side of the irterus. 508 TUMOURS OF THE OVARY The importance of lutein cysts in the ovaries in association with the disease of the chorion known as hydatidiform disease, and especially those cases which have been followed by chorion-epithelioma, are considered in Chap. XL. Fraenkel, A., " Ueber Dermoidcysfcen der Ovarien und gleichzeitige Dermoide (mit Haaren) im Peritoneum." — Wien. med. WocJt., 1883, zxxiii. 865. Grawitz, P., " Casuistische Mittheilungen aus dem Patliologischen Institut zu Greifswald. ii., Dermoidahnliche Cysten im Peritoneum und Diaphragma." — Virchow's AreJi.f.path. Anat., 1885, c. 262. Kolakczek, " Peritonaeale Metastasen eines Eierstocksdermoids und eines Beckensarcoms." — Virchow's Arch. /.path. Anat., 1879, Ixxv. 399. Lucy, R. H., " Double Dermoid Cyst of the Ovaries with rupture into the peri- toneal cavity." — Lancet, 1910, i. 1756. Lawrence, T. W. P., and Randall, M.," Proc. Boy. Soc. of Ilecl., Obstetrical Section, 1908, i. 106. Melchior, Berl. Uin. Woch., 1908, p. 34." Moore, C. H., " Dermoid Ovarian and many Piliferous Cysts ; spontaneous opening of the former at the navel." — Trans. Path. Soc. Loncl., 1867, xviii. 190. Orthmann, H. G., " Ueber Embryoma Tubae." — Mo)iatsschr. f. Geb. und Gyn., 1901, liii. 119. Targett, J. H., and Hicks, H. J., " Two Cases of Malignant Embryoma of the Oxaxj:'— Trans. Obstet. /%^., 1905, xlvii. 287. Wilms, M., "Ueber die soliden Teratome des Ovariums." — ZeigUr's Beit., 1896, xix. 367. CHAPTER L TUMOURS OF THE OVARY (Contm«ed) PAPILLOMATOUS, PAROVAPtlAN, AND GARTNERIAN CYSTS That portion of the ovary wliicli is termed the paroophoron and receives the terminals of the parovarian tubules is usually represented in an adult ovary by a plug of connective tissue which is occasionally referred to as the " tissue of the ovarian hilum " ; it has been the subject of much careful histologic investigation. This tissue is regarded by some writers as the source of the well-known papillomatous cysts of the ovary, and, as epithelial elements occur as residues of the mesonephros in this " hilum tissue," the theory has a mor- phologic basis. In addition, the tissue is probably the source of some of the ovarian fibroids and sarcomas. Papillomatous cysts of the ovary. — These differ from the simple form of ovarian cyst in having their inner walls beset with soft dendritic warts. In the early stages these cj^sts do not affect the shape of the ovary until they attain an important size. The warts vary greatly in number ; some cysts contain few, in others they are so luxuriant as to burst the cyst-wall and then protrude as a cauliflower-like mass. In some the warts will erode the wall at several points, and grow out as soft epithelial buds. Coblenz was the first clearly to distinguish these cysts from those arising in the parovarium and associate them with definite structures. His observations have been confirmed by Doran, who has devoted great attention to this question. The distinguishing feature of these parociphoronic cysts is that they contain warts ; but all ijapillomatous cysts of the ovary are not parooplioronic in origin. It will there- fore be convenient in this chapter to consider the subject of warts in relation to the ovary. A paroophoronic cyst niay 509 510 TU3I0UES OF THE QVABY contain one large tuft surrounded by a few scattered nodules, Avliereas in another example the cavity of the cyst may be so stuffed with them that it bursts. The museum of the Royal College of Surgeons, London, contains an admirable specimen illustrating this. It is thus described in the catalogue : — " An uterus with its appendages. A mass of finely lobulated and pedunculated growths springs from the site of each ovary, the substance of which, with follicles, was discovered on close search at the roots of the growths. These growths Fig. 269. — Ruptured papillomatous cyst (right half of the specimen). {Jfuseuin, Royal College of Surgeons.) were probably enclosed at an early stage in a cyst-wall" (Fig. 269.) There can be little doubt that the opinion expressed in the catalogue, that the growths were probably enclosed at an early stage in a cyst, is correct, for remnants of the cyst-wall remain on the specimen. A distinction must be drawn between rupture of the cyst and perforation of the cyst-wall by the papillomas. In the latter condition cauliflower-like masses of warts project from the surface of the cyst into the abdominal cavity ; some- times at one spot, sometimes in three or four places. When PAPILLOMATOUS GYSTS 511 such cysts burst, the fluid they contain escapes into the belly ; the epithelium derived from the warts infects the peritoneum and leads to a crop of warts. It has been clearly established that when the abdomen has been opened for the removal of a papillomatous cyst the peritoneum has been found studded with warts. A few years later the abdomen has been reopened and all the peritoneal warts have disappeared. Thus they behave like warts on the skin. This fact must be borne in mind, or the operator will hastily assume the disease to be malignant when he finds general peritoneal infection. The disappearance of peritoneal warts after removal of the primary tumour is an interesting fact, and may be probably explained in this way : The life of multiple warts is often very transient, and this is probably the case with peritoneal papillomas ; but as long as the seed supply continues new warts spring up, last for a time, and die, to be succeeded in their turn by a new crop. When the source of epithelium is removed by operation, the warts then existing die, and the crop is not renewed. Exceptionally these papillomatous cysts rupture into the connective tissue of the mesometrium, and I have seen warts clustering around the urachus as high as the umbilicus. The dispersal of the cells from these emancipated warts is no doubt largely effected by movements of the intestines, in addition to the sudden inundation of the belly when the cyst bursts ; but there is an additional complication which not only favours infection but is in itself inimical to life, namely, hydroperitoneum. This condition differs from ascites in the circumstance that it is not the consequence of hepatic, cardiac, or renal disease, but is due to irritation of the peri- toneum by secondary nodules of cancer, warts, tubal disease, and the like. Hydroperitoneum is a constant concomitant of the bursting of a papillomatous cyst. When the cyst is re- moved the exudation ceases. One of the most remarkable examples of hydroperito- neum associated with papillomatous cysts on record is that described by Dr. Pye-Smith. In this case a woman was tapped for hydroperitoneum, between August, 1884, and April, 1894, 299 times. On readmission for the 300th tapping she died. At the post-mortem examination a papillomatous cyst 512 TUMOURS OF THE OVARY was found in connexion with each ovary. These cysts could have been easily removed. The peritoneum was beset with warts. It is important to draw a distinction between epithelial infection, which is such a marked feature of papillomatous cysts of the ovary, and cancerous generalization due to depor- tation of malignant cells by the blood- and lymph- vessels. It is necessary to state that some papillomatous cysts display malignancy by recurring locally. Pozzi has especially em- Tube. Ovarian ligament. Fig. 270. — Euptured papillomatous cyst. phasized the fact that a great number of patients from whom papillomatous cysts are removed make complete and durable recoveries, and I can affirm this from my own experience ; but it is difficult to assert that the recovery is permanent in the face of the following record : Pozzi removed, in 1878, bilateral papillomatous cysts of the ovaries, attended with very abundant hydroperitoneum^ from a woman 25 years of age; recovery was complete for twenty years. In 1898, hydroperitoneum reappeared, and a second operation was performed, but the recurrent tumour could not be removed ; the peritoneal cavity Avas PAPILLOMATOUS CYST'S 5l^ drained, and the patient made a temporary recovery. She died a year later. Pozzi also writes favourably of the ad- vantage of operating, whenever it is possible, on recurrent papillomatous masses, even when they cannot be completely removed, for such a proceeding, accompanied by temporary drainage, is distinctly advantageous to the patient. In 1899 I removed from a woman 40 years of age bilateral papillomatous cysts, and evacuated a large quantity of free peritoneal fluid. In 1905 this patient again came under my observation with a large tense cyst in the hypogastrium as big as her head. I enucleated this cyst ; it contained turbid Fig. 271. — Warty ovary {Nat. size.) A, Ovary; p, parovarium; F, Fallopian tube ; B, mesosalpinx. Two small papillomatous cysts are seen in relation with the tuho-ovarian ligament. fluid and the interior was beset with an abundant crop of soft, but not very vascular, papillomas. She was in good health five years later (1910). Papillomatous cysts of the ovary are most frequent be- tween the twenty-fifth and fiftieth years ; they are the rarest species of cysts which arise in this organ. In most instances they admit of easy removal, but occasionally they burrow deeply between the layers of the mesometrium. In some of these cases it simplifies the operation to remove the uterus with the cysts. So far, in all the cases which have come under my observation the cysts were bilateral. In several patients the disease was much more advanced in one ovary than in its fellow. 2 H 514 TUMOURS OF THE OVARY Warty ovaries. — There is a variety of papillomatous cyst arising in the mesosalpinx independently of the ovary or Gartner's duct. These cysts are usually found near the junc- tion of the tubo-ovarian ligament with the ovarj^, and burrow between the layers of the mesosalpinx (Fig. 271). When fresh they are transparent, and resemble incipient parovarian cysts, but they are unconnected with this struc- ture. The most striking feature of these cysts is the almost invariable presence of a tuft of warts. It is difficult to be sure of the presence or absence of the warts without open- ing the cyst. The warts are composed of very dense fibrous tissue. In this respect they differ in a striking manner from the soft vascular processes found in typical papillomatous cysts. Wart-containing cysts also occur on the free surface of the ovary. PAEOVAEIAN CYSTS The parovarium consists of a series of narrow tubules situated between the layers of the mesosalpinx and closely associated with the paroophoron. It is easily seen, when the mesosalpinx is stretched and held up between the eye and the light, as a series of tubules radiating from the ovary to join a longitudinal tubule situated at a right angle to them. Although the tubules converge as they approach the ovary, nevertheless they remain distinct. Each tubule ends blindly, and is usually lined with epithelium. In form, size, and disposition they resemble the vasa efferentia of the testis. This resemblance was observed by Rosenmiiller, who discovered this structure in 1801 whilst prosecuting ana- tomical researches at Erlangen. The parovarium is homo- logous with the vasa efferentia and epididymis of the testis, for these tubular structures in the male and female are the persistent excretory ducts of the Wolffian body (mesonephros). In the female they are vestigial, whereas in the male they are functional. When present in its typical condition, the parovarium consists of three parts (Fig. 256) : an outer series of tubules, free at one extremity, known as Kobelt's tubes ; an inner set termed the vertical tubules ; and a larger tube running at right angles to the vertical tubules, which may occasionally be PABOVABIAN GT8TS 515 traced downwards to the vagina. This is Gartner's duct ; it corresponds to the vas deferens in the male. The parovarium contains as a rule, twelve tubules ; sometimes as many as seventeen may be counted, and in other specimens as few as five. The cysts which arise in the parovarium are of two kinds ; the more frequent are small pedunculated cysts connected with Kobelt's tubes. They rarely exceed a pea in size, and do not call for much comment, as they are of no clinical importance. They need to be mentioned, however, because they are often confounded with the hydatid of Morgagni. Occasionally some of the vertical tubules will break loose and form pedunculated cysts. Should the cyst rupture, it may be converted into a tuft of fimbriae. The more important cysts are sessile, and remain between the layers of the mesosalpinx. In the early stages it is easy to demonstrate the relation of these cysts to the parovarium. When such a cyst enlarges, it burrows between the layers of the mesosalpinx and makes its way towards the Fallopian tube, which becomes stretched, because the abdominal end of the tube is fastened firmly to the ovary by the tubo-ovarian ligament, and the ovary in its turn is attached to the side of the uterus. In a very large cyst the Fallopian tube becomes greatly elongated, and attains a length of 40 cm. In spite of this extreme stretching, the lumen of the tube is rarely obstructed, and its abdominal ostium can usually be found, the fimbrige being indicated by a few wattle-like processes. Small cysts are, as a rule, transparent, but when they exceed the size of a coco- nut this transparency is lost, and the walls become thick and tough. Small parovarian cysts are lined with columnar epithelium, which is sometimes ciliated ; in cysts of moderate size the epithelium becomes stratified, and in large cysts it atrophies from pressure. Rarely they contain warts. The fluid in a parovarian cyst is limpid and opalescent ; specific gravity 1002 to 1007, reaction alkaline. A substance precipitated by alcohol, is present in large quantity. In big cysts the fluid is often turbid and may contain cholesterin. When parovarian cysts burst the fluid is quickly absorbed, and excreted by the kidneys. 516 TUMOURS OF THE OVARY The points which enable a large parovarian cyst to be dis- tinguished from an oophoronic cyst are these :— 1. The peritoneal coat is easily stripped off. 2. The ovary is usually found at the side of the cyst. 3. The cyst is generally unilocular. 4. The Fallopian tube is stretched over the cyst, but does not communicate with it (Fig. 272). 5. The specific gravity of the fluid does not exceed 1010, and may be much lower. 6. In some specimens the tissue of the mesosalpinx is greatly thickened. It was formerly believed that cysts originating in the Fia 272,— Cyst of the parovarium, showing its relation to ovary and tuhe A, Oophoron ; b, paroophoron ; r, Fallopian tube. (| nat. size.) parovarium rarely exceeded the size of an orange, but Bantock demonstrated that parovarian cysts may attain very large proportions, and be capable of containing several litres of fluid. The largest parovarian cyst which has yet come under my care contained 20 litres of turbid fluid. The age at which parovarian cysts occur is of some in- terest. It has already been mentioned that cysts of the oophoron are encountered at any period, from foetal life up to extreme old age. The occurrence of a parovarian cyst has not, as far as I am aware, been recorded in an individual before the age of 16; many undoubted cases have been observed at 17, 18, and 19, the cysts being large enough to rise above the pubes. Before 16 the parovarium appears PABOVABIAN CYSTS 517 to be quiescent, but on tbe advent of puberty it seems to undergo great stimulation ; a very large proportion of cysts, generically classed as ovarian, removed between the ages of 17 and 25, arise from it. rig. 273.— Ovary and stump of 111, J 3ft after axial rotation, ending in complete detachment of a parovarian cyst. * The rounded stump of the tube at the point of detachment. Parovarian cysts do not often contract adhesions, even when they suppurate. The layers of the mesometrium stretched over them occasionally contain an unusual pro- 518 TUMOURS OF THE OVARY portion of unstriped muscle-fibre ; they rarely suppurate. Like other forms of cysts and tumours related to the ovary, they are liable to axial rotation and complete detachment (Fig. 273). GARTNERIAN CYSTS. A large experience of ovarian and parovarian cysts has served to convince me that many papillomatous cysts have an origin independent of the paroophoron. A careful study Gaitnei's Duct Fig. 274. — Uterine segment of a cow's vagina, showing two large cysts developed in the terminal segment of Gartner's duct. {Museum, Royal College of Surgeons.) of the relations of these cysts shows that many of them burrow deeply by the side of the uterus, and even extend along the wall of the vagina. It is known that Gartner's duct occasionally persists in women, and after leaving the parovarium it traverses the layers of the mesometrium and runs down the side of the uterus to reach the vagina. As it approaches the cervix, it is often embedded in its tissue. Evidence is slowly accumulating in support of the opinion GARTNERIAN GY8TS 519 that some papillomatous cysts of the mesometrmm, especially those which burrow deeply by the side of the uterus, arise in persistent portions of Gartner's duct, near its termination. Cysts arising in the lowest segment of this duct occasionally bulge into the vagina. The cystic tendencies of Gartner's duct can easily be studied in cows (Fig. 274). In these animals the ducts are sometimes as large as crow-quills. Usually they become gradually lost on the sides of the uterus, but occasionally they may be traced to the vagina. Bantock, G. G., " On the Pathology of certain so-called Unilocular Ovarian Gjsts."—l'rans. Obstet. Soc. Land., 1873-74, xv. 105. Coblenz, H., " Zur Genese und Entwickelung von Kystomen im Bereich der inneren weiblichen Sexual organe." — Virchow's Arch. f. path. Anat., 1881, Ixxxiv. 26. Doran, A. H. G., " Proliferating Cysts in the Ovary of a seven-months Foetus." —Trans. Path. Soc, 1881, xxxii. 147. Pye-Smith, P. H., " Papillomatous Tumours of both Ovaries." — Trans. Path. So3., 1893, xliv. 111. CHAPTER LI TUMOURS OF THE OVARY (Continued) OVARIAN FIBROIDS; SARCOMAS AND CARCINOMAS Ovarian fibroids. — Tumours are occasionally met witli in the ovary which in their naked-eye and microscopic char- acters are indistinguishable from the very hard variety of uterine fibroid. In their most typic form they are easily re- cognized, being ovoid in shape, regular in contour and smooth ; intensely hard, encapsuled and, as a rule, free from adhesions. On section the fibrous tissue displays the whorled arrange- ment which is such a conspicuous feature of the hard variety of uterine fibroids. When the tumour is divided in such a way as to include the ovarian ligament, a small portion of the ovary may usually be detected associated with the liga- ment (Fig. 275). Yirchow described and figured this re- lationship of the ovarian fibroid to the ovary in 1867. Ovarian fibroids sometimes soften, and this leads to the formation of spurious cysts in their substance. Another feature of some importance, and one already mentioned, is their intense hardness ; and in some cases this is so obvious that it has enabled me to suspect the nature of the tumour before operation. Ovarian fibroids are prone to calcify ; Hand- field-Jones removed a calcified tumour of this kind from a woman aged 19. They are occasionall}^ complicated with hydroperitoneum, and, except in this circumstance, they rarely . produce any very obvious impairment of the general health. Ovarian fibroids are as a rule unilateral, but I have in one instance found both ovaries affected, and they may occasion- ally be associated with uterine fibroids ; of this companion- ship I have seen one example. It is also usual for these tumours to be encaj)suled in the ovary, no matter what size they attain ; but there are examples which grow within the ovary and project from its surface like subserous fibroids of .520 OVARIAN FIBROIDS 521 tlie uterus. It is probable that some tumours known as corpora fibrosa (Patenko), supposed to arise from corpora lutea; are of this nature. However much ovarian fibroids may resemble uterine fibroids in possessing definite capsules, and in their gross as well as minute structure, and the extraordinary vortex-like arrangement of the constituent tissues^ they differ in a very marked way in their age-distribution. Uterine fibroids only Ovary Fig. 275. — Ovariau fibroid in longitudinal section. 30 years of age. {NfcL size.) From a patient arise during menstrual life, which in its widest sense gives them an age-limit of thirty years (15 to 45), but ovarian fibroids arise in advanced life. In fourteen cases under my own observation the youngest patient was 27 and the oldest 73 ; in one patient the tumour had undergone axial rotation and twisted its pedicle. One tumour complicated pregnancy and was successfully removed. McCann removed a tumour of this kind from a woman 73 years of age ; she was well four and a half years later. 522 TUMOURS OF THE OVAEY The earliest age at which these tumours have been ob- served is the twentieth year. This was a case reported by Doran, in which the patient married at 15, but had borne no children. Previously to the removal of the tumour by Knowsley Thornton (1884) sexual desire appears to have been absent. After recovery, this instinct rapidly developed ; the patient left her husband, and bore a child to another man. Ultimately she returned to her home in good health. Baillie (1799) gives a good figure of an ovarian fibroid, and writes " that it resembles exactly in its texture " uterine fibroids, and draws attention to its rarity. Ovarian fibroids occasionally complicate pregnancy and have led to Csesarean section. One of the greatest difiiculties in connexion with our knowledge of the solid tumours of the ovary, especially the variety termed fibroma, has been the absence of information concerning the after-history of those patients who have been submitted to operation. This defect has been removed by the publications of Doran, Briggs and Fairbairn. I have followed up the after-history of ten patients' under my care. Nine were alive at intervals varying from one to six years after operation. One died three months after the operation from a chronic affection of the lung and pleura. The results of careful inquiries into the after-history of patients who have had ovarian fibroids removed, establish clearly that these tumours are as innocent as the hard variety of uterine fibroids. The immediate result of the removal of such tumours is excellent, even when associated with hydroperitoneum. Sarcoma of the ovary. — The ovary, like other paired organs^ is very prone to become the seat of sarcoma in early life : to this succeeds a period of comparative immunity, followed by a second period of renewed but diminished liability. Ovarian sarcomas in girls differ in several points from those found in women, and their histologic peculiarities are such that I proposed the term oophoromas for them ; they attack both ovaries in about half the cases, grow very rapidly, often attain formidable proportions, and quickly destroy life. 8AB.G0MA OF THE OVARY 523 Their removal is attended with an excessively high rate of mortality, and, in the patients who recover, quick recur- rence is the rule. In structure they consist of round or spindle cells, in which collections of cells are often conspicuous, resembling the alveolar disposition of cancer. This appearance is due to the entanglement of ovarian follicles in the sarcomatous tissue. Sarcomas are rare in the ovaries between the sixteenth and twentieth years ; after this age they are encountered occasionally, and as a rule are unilateral. The two common periods for sarcoma to arise in the adult ovary are from the twentieth to the thirtieth years, and after the meno- pause. The hard encapsuled tumours of the ovary formerly classed among the sarcomas are now described as ovarian fibroids, and are free from the odium of malignancy. Many ovarian sarcomas arise quickly, attain very large proportions often in a few months, and are accompanied by hydroperitoneum and marked leucocytosis. Such tumours are very soft and succulent, and occupied by spurious cysts due to degenerative changes. Microscopically they consist of round or oat-shaped cells, and, as a rule, the more these cells predominate the more ominous is the outlook for the patient. Often the cellular elements burst the limiting tissues of the tumour, and, implicating adjacent organs — such as uterus, bowels, bladder, veins, and arteries — render removal during life impossible. Dissemination also occurs, and life is often destroyed within a few months of the onset of symptoms. Some of these rapidly-growing tumours of the ovary are described by German writers as endotheliomas. Ovarian sarcomas are more common in children than in adults, and have been observed even in a foetus (Doran). I collected from current literature one hundred cases of ovariotomy in girls under 15 years of age. Of this series forty-one were simple cysts or adenomas, and thirty-eight were typical dermoids ; the remaining twenty-one being sarcomas. This, however, is far short of the real propor- tion of sarcomas, because there are many records in which no operation was undertaken, the descriptions being based on post-mortem examinations. Another important feature was the heavy mortality 524 TUMOURS OF THE OVABY among the patients with sarcomas submitted to operation. Seven out of the twenty-one patients died, and of the four- teen who recovered I was able to ascertain that four died from recurrence within a year of the operation. (The tables are furnished in my work, " Surgical Diseases of the Ovaries," second edition, 1896.) The youngest child on record who has been operated on for sarcoma of the ovary Avas one of 33. months. Un- fortunately, death occurred a few hours after the operation. (Hoffman.) Carcinoma of the ovary. — The observations of Schla- genhaufer, Cuthbert Lockyer, Briggs and Walker, and my own, show that many large tumours of the ovaries, unilateral and bilateral, which exhibit the structure of cancer and were formerly regarded as arising primarily in the ovary, are in reality secondary to cancer situated in some other organ. The relationship of the ovarian masses to cancer in some other organ is demonstrated by the fact that the minute structure of the tumour in the ovary varies according to the situation of the primary focus. Thus, when the infec- tion of the ovary is secondary to a cancer somewhere in the gastro-intestinal tract, the ovarian tumour presents the features of gastric or colic cancer. When the disease is secondary to carcinoma of the breast, it will have the characters of mammary cancer. The common situations for cancer liable to infect the ovary are the gastro-intestinal tract, the gall-bladder, the breast, the uterus and Fallopian tube. In my early observations I thought it was only the solid tumours of the ovaries that could be placed in this category, but a longer experience has taught me that if a woman whose peritoneum is invaded by cells from a cancerous lump in the breast, stomach, or colon has an ovarian cyst in her pelvis and the cancer-particles become implanted on it, they give rise to a malignant cystic tumour of the ovary. This occurs more frequently than published records would lead us to suspect. Moreover, in some of the cases where an ovarian cyst co-exists with primary cancer of the Fallopian tube, the cyst may become cancerous by implantation, as explained in Chap, xxxix. GARGINOMA OF THE OYAEY 525 It is astonishing to find what a large number of these cases are missed. Owiriotomy, unilateral and bilateral, has been often performed and the primary cancer in the stomach or intestine overlooked, although the patient has exhibited the signs common to these conditions, such as pain, vomit- ing, and progressive emaciation. In cases of bilateral tumours of the ovaries accompanied by vomiting and ascites, a careful examination of the abdominal viscera, Fig. 276. -Ovarian tumour in section. It consists of nodules of carcinoma, primary growth arose in the colon. The especially the stomach, should be made. Primary foci of malignant disease in the gastro-intestinal tract are easily overlooked unless especially sought, either because they often occupy a position that is not exposed in the course of an operation, or on account of their small size. On several occasions I should have missed a primary cancerous focus in the course of an ovariotomy unless I had deliberately examined the gastro-intestinal tract. It is a significant fact that, whenever surgeons have made inquiries into the remote results of ovariotomy, they have been astonished to find that many of the patients have perished from recurrence in 526 TUMOURS OF THE OVARY the abdomen, or from intestinal obstruction. The fact that many of these large tumom^s of the ovaries are due to implantation of cancerous cells from a primary focus in another organ offers a satisfactory explanation of many such unhappy sequences. Handley has demonstrated, in a most convincing manner, that cancer of the breast spreads by permeating the deep fascia ; by an insidious process the cancer-cells slowly creep along the lymphatics of the fascial plexus until they reach the epigastrium immediately below the ensiform cartilage : at this point the cancer-filled lymphatics of the fascial plexus in the middle line are separated from the sub- peritoneal fat only by a simple layer of fibrous tissue. Through this weak defence the cancer-cells slowly find their way into the general peritoneal cavity and engraft themselves on the omentum and other suitable visceral plots, whereon they thrive and grow into metastatic nodules, or lumps. Many of these infecting cells are conveyed into the pelvis, and lodge on ovary. Fallopian tube, uterus, or pelvic peritoneum. The fluid normally present in the belly serves as an admirable vehicle for the transport of such cells, easily enabling them to reach the pelvic recesses, where they remain undisturbed and grow into deadly masses. If we apply Handley's observations on the ser- pigmous spread of mammary cancer to a primary cancer in the stomach, gall-bladder, or colon, we may read its course in this way : Arising in the mucous membrane, it slowly permeates it and implicates the submucous, mus- cular, and peritoneal coats ; the cancer-cells can then escape freely into the great serous cavity and be distributed by the fluid, aided by the movements of the bowels, and gradu- ally reach the pelvis and other abdominal recesses. In the pelvis the most obvious organs on which they could fall would be the ovaries, as these so often rest on its floor. Under such conditions the ovaries may be fairly pictured in the mind as receiving a covering of falling cancer-cells, as evergreen shrubs are clothed by snowflakes in winter. The fact that other parts of the internal genitalia receive these cells is a matter of some importance, because one of the most striking features of operations for the removal of GAEGINOMA OF THE OVARY 527 malignant ovaries is the rapidity with which the disease recurs. Lockyer made a valuable observation relating to this : he examined microscopically the tumours removed for bilateral carcinomatous disease, and although the attached tube and mesosalpinx belonging to each tumour appeared normal to eyes and fingers when examined macroscopically, they were found extensively infected with cancer through the lymphatics. Under these conditions I have found one ovary rig. 277. — Cancerous ovary, secondary to cancer of the colon, in section. The Fallopian tube is infected by the cancer. From a woman aged 56 years. as large as an orange, due to secondary cancer, and its companion smaller than normal, yet on microscopic examin- ation it also was permeated with cancer. Treatment. — The outcome of these pathological observa- tions will determine those who have to deal with bilateral malignant tumours of the ovaries to examine carefully the patient for evidence of primary cancer in the gastro-intestinal tract. If the disease is in such a position that it can be excised with good prospect of success, this may be done. Then it will be necessary to remove not only the infected 528 TUMOURS OF THE OVARY ovaries but the tubes, adjacent segments of the mesometria, and the uterus. In many instances the removal of these large ovarian masses is urgently needed in order to make the patient comfortable. Operations of this kind can only be carried out with hopeful prospects when there is no other evidence of gross infection than that afforded by the ovaries. Baillie, M., " Morbid Anatomy of the Human Body," London, 1799, PI. vii. Bland-Sutton, J., "A Clinical Lecture on Secondary (Metastatic) Carcinoma of the Ovaries."— Srit. Med. Journ., 1906, i. 1216. Bland-Sutton, J., "Cancer of the Ovary." — Brit. Med. Journ., 1908, 1. 5. Briggs, "Fibroma of the Ovary." — Brit. Med. Journ., 1897, i. 1083. Doran, A., " Fibroma of the OvnYj."~Tra7is. Obstet. Soc, xxxviii. 187. Doran, A., " Large Ovarian Tumours in a seven-months Child." — Trans. Path. Soc, xl. 200. Fairbaim, J. S., " Fibroid Tumour of the Ovary." — Trans. Obstet. Soc., 1903, xliv. 177. Handfield-Jones, M., "Calcified Ovarian Fibroma." — Trans. Obstet. Soc, 1906, xlviii. 332. Handley, W. S., " Dissemination of Mammary Cancer." — Brit. Med. Journ., 1905, i. 663. Lockyer, Cuthbert, " Carcinoma in the Muscular Wall of the Uterus Secondary to Cancer of both Ovaries." — Trans. Obstet. Soc. Lond., xlvi. 302. Schlagenhaufer, Fr., " Ueber das metastische ovarial Carcinom nach Krebs des Magens, Darmes und andere Bauchorgane." — MonatscU. f. Geb. und Gyn., Berlin, 1902, xv. 485. Virchow, R., " Die Krankhaften Geschwtilste," 1867, iii. 224. CHAPTER LII TUMOURS OF THE OVARY (Conceded) AXIAL KOTATION OF OVARIAN TUMOUES : EPITHELIAL INFECTION AND SUPPURATION OF OVARIAN CYSTS. In 1865 Rokitansky drew attention to the fact that ovarian and uterine tumours sometimes rotate and twist their pedicles, or drag upon them in such a manner as to compress the vessels traversing the pedicles, the proper nutrition of the tumours thus being interfered with. Occa- sionally the torsion, and in some instances also the tension on the pedicle, lead to complete detachment of the tumour. He also drew attention to the fact that certain abdominal viscera are apt to undergo axial rotation. Of recent years much attention has been devoted to this subject, and accumulated observations have served to show that almost every variety of abdominal tumour and all the viscera, except the liver, are liable to this accident. That a tumour hanging freely in the belly should, by mere alteration of the position of the body, or by motion im- parted to it by a tumult of the bowels, spin round and twist its pedicle is as comprehensible as the fact that a good weathercock moves in varying directions under the influence of the wind. It is, however, puzzling to find the spleen, dis- tended Fallopian tubes, undescended testes, the pregnant uterus, the kidneys, the ceecum, and the stomach hable to similar rotation. Ovarian tumours of all kinds are very liable to axial rota- tion, even in the newly born (Otto von Franque). Concerning its cause very little is known. Various explanations have been advanced. It has been attributed to the alternate distension and evacuation of the bladder (Klob), or to the passage of fseces through the rectum (Lawson Tait) ; to sudden move- ments, such as a fall, slip, or unusual exertion (Thornton). 2 I 529 530 TUMOURS OF THE OYABY An important fact to remember is the frequency with which this accident occurs when ovarian cysts comphcate preg- nancy, and especially when an ovarian cyst complicates a myomatous uterus large enough to fill the pelvis. When both ovaries are converted into cysts the risk of twisting is nearly the same as when pregnancy and an ova- rian cyst are associated. When both ovaries are cystic and pregnancy ensues, the risk of axial rotation is more than doubled. The torsion may occur early in pregnancy or be delayed till delivery or miscarriage. In one instance at least, in a case of bilateral ovarian dermoids, both tumours had twisted their pedicles (Doran). The occurrence of acute torsion immediately after de- livery is due to the rapid diminution in the size of the uterus and to the movement which this organ, as it sinks into the pelvis, imparts to the tumour. In a case under my own ob- servation, acute axial rotation of an ovarian cyst as big as a fist was caused by the movement and dragging of a prolapsed uterus : the rotation caused the tumour to be impacted low in the pelvis, and the uterus remained outside the vagina and could not be reduced until the tumour had been removed. Rotation of a cyst in the early stages of pregnancy is probably due to the gradual enlargement of the uterus dis- placing the tumour upwards ; and as the pressure is exerted upon one side of the cyst, it would be in a favourable position to impart a rotatory motion to a non-adherent cyst. The amount of rotation varies greatly. In some cases the cyst has only turned through half a circle ; in others as many as twelve complete twists have been counted. The direction of the rotation may be from right to left, or vice versa, but cysts exhibit a strong tendency to rotate towards the middle line rather than from it. Tumours of the right and the left side are equally liable to torsion. Small tumours twist more freely than large ones, and a long and slender pedicle favours rotation. The force with which some of the large cysts rotate is very great, for in some instances the uterus is involved in the twist. In one remarkable case the ovary was caught in the pedicle of a parovarian cyst during rotation and was divided (Fig. 278). The effect of torsion on the circulation depends on the AXIAL ROTATION 531 tightness of tlie twist, and this varies with the thickness of the pedicle. The vessels in a long thin pedicle would suffer obstruction more quickly than those in a short thick one. When a pedicle is torsioned the thin-walled veins become compressed, whilst the more resilient arteries continue to convey blood to the cyst. The result is severe venous en- gorgement, and this leads to extravasation of blood into the cyst- wall ; in many cases the veins rupture, and haemorrhage takes place into the cavity of the cyst. The haemorrhage rig. 278. — Parovarian cyst which rotated and twisted its pedicle. The ovary was caught in the pedicle and divided. (^Comyns Berkeley. ) may be so profuse as to cause profound anaemia, and even death. When the venous circulation is completely arrested in con- sequence of torsion, the appearance of the cyst is very striking and characteristic. On the abdomen being opened during life, instead of the cyst presenting the famihar white glistening appearance, it has a deep, dark, lustreless hue, which is most intense near its attachment to the pedicle. In milder degrees of torsion the change in colour only affects the base of the tumour. The pedicle on the distal side of the twist presents 532 TUMOURS OF THE OVABT the same dark hue, but on the uterine side it is, as a rule, of natural tint. The contrast of colour in the two parts of the pedicle is very striking. The walls of the cyst are thick and succulent ; the blood contained in the cavity, or in the loculi if multilocular, may be of a chocolate or of a dark-red colour. When such a cyst is removed from the body and the blood is allowed to drain away, or is washed away by a gentle stream of water, the tissues will resume their natural colour. This should be remembered, because some writers have attributed the dark colour to gangrene of the cyst. This is erroneous ; gangrene of an ovarian cyst is a rare event, and can only take place when air is admitted from without, as during the operation of tapping, or when intestinal fluids obtain access to it. The usual effects of acute torsion of the pedicle are passive congestion, thrombosis, extravasation of blood into the tissues of the tumour, and necrosis. Necrosis is localized death, in contrast to the death of the organism as a whole, or " somatic death." Moist gangrene is necrosis followed by decomposition and putrefaction of the dead tissues. When soft parts necrose in situations where they are accessible to putrefactive organ- isms, such as the exterior of the body, the lungs, or the intestinal tract, decomposition rapidly ensues, especially if the parts contain much blood. In the case of ovarian tumours with twisted pedicles, not in communication with the outer air directly or indirectly, micro-organisms rarely gain access to them. It is therefore erroneous to describe as gangrene the changes observed in cysts with torsioned pedicles. This is further illustrated by the circumstance that small ovarian tumours may be completely twisted from their pedicles and subsequently shrink. Were the changes in the cyst gan- grenous in character, general infection of the peritoneum and death would be the inevitable consequences. Burden Sanderson, in his article on the Pathology of Inflammation, refers to the peculiar plan of emasculating animals known as bistournage. In this method no instrument is used; the testicle is freed from its association with the dartos, then twisted on the spermatic cord as on an axis, four AXIAL ROTATION 533 or five times, the whole manipulation being performed with prodigious rapidity. If the animal is killed afterwards and Fig. 279.— Ovarian dermoid containing hair and grease which had twisted its pedicle many times. * The stiimp of the Fallopian tube. the arteries are injected, it is found that no blood enters the spermatic artery beyond the twisted part of the cord. Con- sequently, while the surrounding parts receive their natural 534 TUMOURS OF THE OVABY supply of blood from the piidic artery and preserve their vitality, the testicle itself is irretrievably condemned to death. .We have in the above method practically a crucial experiment, which demonstrates that when a testicle is deprived of blood in consequence of axial rotation it necroses and finally atrophies. A perusal of the records of cases described as gangrenous cysts indicates that the reporters have regarded the deep livid hue of such cysts as evidence of gangrene, and that others have confounded suppurating with gangrenous cysts. Rotation of an ovarian cyst, when it gives rise to such severe changes as have just been considered, may be described as acute torsion. It frequently happens that during the performance of ovariotomy a thick pedicle is found twisted through half or even a complete circle, without producing an appreciable effect upon the tumour. In others, torsion takes place so gradually, yet so completely, that the pedicle is twisted like a rope, and not infrequently the pedicle breaks and the tumour becomes detached from its uterine connex- ions. To this variety the term slow or chronic torsion may be applied. Its effects are not less interesting than those which follow acute twisting. When rotation occurs slowly, the walls of the cj^st inflame and adhesions are established between the cyst and the omentum or the parietal peritoneum; such adhesions become vascular and maintain the vitality of the C3^st-wall after circulation is arrested through the pedicle. Cysts have been observed in all stages of transplantation. Acute torsion is more frequent in tumours of medium size ; it also occurs in smaU cysts; but it is the small tumours, especially dermoids, in which slow torsion takes place. The dermoid which had undergone axial rotation (Fig. 279) had so lengthened its pedicle that the tumour sometimes rested in the loin and resembled a very movable kidney : it produced no pain, but annoyed the patient by its excursions about the belly. The symptoms of acute torsion of an ovarian cyst are often so characteristic as to lead to a correct diagnosis. When a woman complams of sudden and violent pain in the abdomen, accompanied Avith vomiting, and she is known to have an ovarian tumour, or she presents herself for the first time to MODES OF DEATH 635 the surgeon and these signs are associated with an abdominal swelling the physical signs of which are indicative of an ovarian tumour, axial rotation should be suspected. Should the patient possess a gravid uterus as well as an ovarian cyst, it is even more probable that rotation has occurred ; or if she has an ovarian tumour and has been recently delivered, this is an additional reason for suspecting that the symptoms arise from a twisted pedicle. Clinical observations demonstrate that the predominant signs of acute axial rotation of abdominal tumours and viscera are those common to a strangulated hernia minus stercoraceous vomiting. Even the presence of fsecal vomiting does not always negative the existence of acute axial rotation of an ovarian tumour, for a loop of bowel is sometimes involved in the twist and produces intestinal obstruction. Suppuration in ovarian dermoids. — When air or intestinal fluids gain access to these tumours, then septic infection with all its attendant evils is the result, and unless the pus finds an outlet the individual dies. The pus in a suppurating dermoid sometimes bursts into the bowel, bladder, vasfina, or through the abdominal wall at or near the umbilicus. When the cyst communicates with the bladder it will sometimes entail very great misery, because fragments of bone, teeth, locks of hair, and sloughs become impacted in the urethra. Cystitis is an almost constant accompaniment. Ovarian teeth in the bladder have formed the nuclei of phosphatic calculi. Hair from ovarian dermoids entering the bladder is voided with the urine, a condition of things described by French surgeons as inlimiction. Rupture. — Ovarian cysts of all kinds are liable to burst into the belly, either without any obvious cause (spontaneous rupture), or from violence, such as falls, blows, coughing, vomiting, the manipulation of physicians, or an immoderate fit of laughter. Modes of death. — Tumours of the ovaries are now so promptly removed when discovered that there are happily few opportunities of studying the ways in which they destroy life. The chief modes are the following : (1) Pressure on 536 TUMOURS OF THE OVARY the ureters, leading to hydronephrosis and uraemia : (2) cystitis and pyelitis ; (3) intestinal obstruction ; (4) suppura- tion of the cyst and septic infection from leakage (peritonitis); (5) haemorrhage from rupture of the cyst ; (6) axial rotation of the tumour; (7) impediment to delivery; (8) epithelial infection of the peritoneum, and occasionally dissemination (malignancy). Treatment. — All ovarian cysts and tumours should be removed entire at the earliest possible moment. von Franque, Otto, " Ovarialcyste mifc Abdrehung des Stiels beim Neuge- borenen." — Zeitschr. f. Geb., 1900, xliii. 257. Rokitansky, Prof., " Ueber der Strangulation von Ovarialtumoren durcb Achsendrehung." — Zeitschr.der K.K. GeseUscliaft der Acrzte inWien, 18G5. Sanderson, Burdon, "Pathology of Inflammation." — Holmes and Hulke's " System of Surgery," i. 84. CHAPTER LIII TUMOURS OF THE MALE GENITAL GLAND (TESTICLE) Even when divested of what may be called its adventitious tunics, acquired as a result of its emigration from the abdominal cavity to a position in the pouch of skin called the scrotum, the testis is a complex gland, for its ducts, the vasa efFerentia, epididymis, and vas deferens, were originally the excretory ducts of the mesonephros (Wolffian body). A Paradidymis. Kobelt's tubes. Fig. 280. — Diagram to show the relation of the mesonephros and its ducts to the adult testis. study of the evolution of the male secretory organ of verte- brates indicates clearly enough that the ducts have under- gone a change of function, and their relation to the testicle is secondary. An examination of the embryonic testis shows that remnants of the mesonephros persist among the ducts, and only a few of the Wolffian tubules are utilized by the testicle. 537 538 TUMOURS OF THE TESTIS The relation of the various embryonic structures to each other is shown diagrammatically in Fig. 280. In the adult it will be seen that a few of the Wolffian tubules become the vasa eff'erentia, the remainder usually atrophy ; but in many individuals one, two, or more persist, usually as pedunculated cysts of small size at the top of the testicle. The shrunken remains of the mesonephros (Wolffian body) sometimes persist as a collection of cfecal tubes furnished with epithelium, lying among the vasa efferentia . The secreting tibsue of the testis. Fig. The tumour. 281. — A testis in section. The paradidymis is replaced by an adenoma. The secreting tissue is a flattened band at the upper pole of the tumour. between the epididymis and the testis, and often extending a little distance into the tissues of the cord. These remnants are known as the paradidymis. Thus, in the male the mesonephros is represented by the paradidymis, its tubules by the vasa efferentia and Kobelt's tubes, and its ducts by the epididymis and vas deferens. The terminology of tumours of the testis is very confusing; in this work an interesting group will be considered under the title of Cystic Disease of the Testis, a phrase introduced by Curling (1853). This surgeon pointed out an important feature connected with these tumours, namely, that they CYSTIC DISEASE 539 rise in the tissues of the rete testis, a vestigial structure lying between the body of the testis and the epididymis. As the tumour increases in size the secreting tissue of the testis is flattened into a mere stratum over its upper pole. There are three varieties of tumour arising in the paradidymis, and in their most specialized forms they have received distinctive names — adenoma testis ; cystic disease of the testis ; and Fig. 282. — A testis in section : it is the seat of a cystic tumour which arose in the paradidymis. * The secreting tissue of the testis. (3Iuseum, Royal College of Surgeons.) dermoid or teratoma of the testis. There are intermediate forms, and specimens occasionally come under observation in which the characters of the three varieties are blended. Adenoma testis in its typical form (Fig. 281) is a solid tumour composed of epithelial tubules with narrow lumina ; these epithelial cylinders lie in juxtaposition without the intervention of connective tissue. Tracts of hyalin cartilage are sometimes present. Here and there, in apparently solid Fig. 283.— A testis with the spermatic cord and the spermatic vessels, removed by- radical orchidectomy from a man 31 years of age. The testis is shown in section. A cystic tumour has grown between the body of the testis and its epididymis. The secreting tissue of the testis is flattened over the upper pole of the tumour. 540 CYSTIC DISEASE OF THE TESTIS 541 tumours, cysts due to the dilatation of the tubules are seen in the sections. The cystic form is a very striking tumour. On section it is seen to be made of cystic spaces greatly varying in size : some are no larger than a rape-seed, and others may attain the size of a hazel-nut (Fig. 282). Many are distinctly tubular, and the cysts sometimes communicate with each other. The cysts are lined with epithelium, which may be columnar, cubical, or stratified. In some specimens occurring in boys, striated muscle-tissue has been detected. Fig. 284. — A lymph-node from beneatli the left clavicle enlarged secondary to a cystic tumour of the testis. Cystic disease of the testis is sometimes malignant and infects the lymph-glands. In 1909 I removed the right testis of a man 31 years old by radical orchidectomy, and at the same time removed an enlarged lymph-node lying on the inferior vena cava. This gland was examined microscopically ; it contained a collection of small cystic spaces lined with epithelium identical with that in the testis (Fig. 283). Six months later the man again came under observation with a cystic tumour in the left side of the neck under the sternal end of the clavicle. This was removed ; on section it appeared transformed into cysts (Fig. 284). These were lined 542 TUMOURS OF THE TESTIS with epithelium like that found in the testis. The infection must have travelled up the thoracic duct. A year later he was in good health. The dermoid or teratomatous tumour of the testis is very rare, and as striking in its general naked-eye features as the cystic form, for it contains hair and teeth {Fig. 285). It is the rarest form of all tumours of the testis. In order to give some idea of its rarity, it may be mentioned that during the last thirty years only five examples have been Fig. 285. — Embryoma of the testis in section. From a Chinese boy aged 19, in whom it was congenital. {Muse/on, Royal College of Surgeons. ) recorded by British surgeons : D'Arcy Power, 1887 ; Jackson Clarke, 1896 ; Bland-Sutton, 1903 ; Kuhne, 1908 ; and Barrington, 1910. The sources of these tumours are inter- esting. Clarke's specimen was removed from a Hindu by Lt.-Colonel C. M. J. Giles ; my specimen was removed by R. T. Booth from a Chinese student in Hankow (Central China) ; and Kuhne's came from a Chinese boy aged 4 at Tungkau (China). The cavity of the teratoma (Fig. 285) contained the usual " embryonal rudiment " embedded in sebaceous matter and loose hair. The rudiment was composed of bone, hyalin BJSRMOIDS 543 cartilage, and a miilticuspidate tooth. The testicular tissue formed a flattened stratum outside the wall of the cyst. In its gross anatomy and structural details this tumour reveals the usual features of dermoids growing in relation with the testis. Some, it is true, are more complex and con- tain nerve-cells, as in one very carefully reported case examined by Cornil : in a "bud" growing from the cyst- wall a collection of nerve-tissue containing ganglion-cells was detected. In its clinical details the tumour from the Chinese boy did not differ from its forerunners. In nearly all the recorded cases enlargement of the testis was observed at or shortly after birth. The tumour seems to have caused little inconvenience to these boys ; indeed, it appears to lie dormant till puberty, then bruises and knocks, or abscesses and sinuses, cause trouble and lead to surgical interference. Recent observations show that testicular teratomas are even more complex than these facts indicate, for Schlagen- haufer discovered that some of these tumours contain tissue indistinguishable microscopically from that found in the typical chorion-epithelioma. These observations have been confirmed by other pathologists. Most of our knowledge of testicular dermoids dates from an elaborate article published by Yerneuil in 1855, founded on the reports of nine cases he collected from the literature of the preceding one hundred and fifty years, and one example which came under his own observation. The con- clusions expressed in this admirable paper have become classical, and form the foundation of our knowledge of the subject ; and even at this date, nearly half a century since its publication, Verneuil's views are reproduced (frequently without any reference to, and often perhaps in ignorance of, their source) in monographs devoted to diseases of the male genital organs and in text-books of surgery. It is true, not- withstanding the fact that these tumours can now be studied with all the advantages of modern histologic methods and differential staining, that we know no more concerning their pathogenesis than Verneuil knew ; and testicular dermoids re- main with us, as they were with him, pathological curiosities. It is also noteworthy that dermoids of the testis, accordmg 544 TUMOURS OF THE TESTIS to all the available records, are unilateral, whereas ovarian dermoids are very frequently bilateral. Verneuil shows in the title of his paper (" Memoire sur rinclusion scrotale et testiculaire ") — which title, he relates, was selected as conveying precisely the view he held in regard to the nature of the disease — that he believed testicular dermoids belongfed to the class of double monsters known as Fig. 286.— Undescended testis removed from a colt. It is associated with a large dermoid containing grease and coarse hair Uke that of the mane and tail. parasitic foetuses. A study of the records published during the last ten years supports Verneuil's contention that der- moids within the tunica vaginalis, though attached to and often intimately associated with the testis, are not really " of the testis " in its strictest sense : they do not arise from transformation of testicular tissue. In some of the cases the dermoid was attached to the gland by such slender con- nexions that the surgeon succeeded in detaching the tumour and preserved the testis. Admirable conservative operations DERMOIDS 545 of this character are recorded by Cornil and Berger, Chevassu, and Rechis. These typical dermoids differ from the adenomatous and cystic forms in being benign. Horses are especially liable to testicular dermoids, a fact known to Verneuil; and they are often associated with unde- scended testes. Like typical ovarian dermoids, they possess an ill-developed embryonal rudiment contained in a cyst, covered with pilose skin and stuffed with loose hair, grease, and occasionally teeth resembling equine incisors. The hair resembles that of the mane or tail (Fig. 286). The relation Fig. 287. — Hyalin cartilage in the stroma of a recurrent carcinoma of the rectum. {After Foulerton.) of the dermoid to the paradidymis is the same in horses as in man. The occurrence of dermoids in the undescended testes of horses has a clinical interest, for, as I have already mentioned, in the records of the human cases, although the unusual size of the testis was invariably noticed at birth, yet it did not interfere with the descent of the organ. There is a case recorded by Delbet in which a testis, retained at birth in the inguinal ring, gradually descended to the scrotum ; subse- quently it was found to be occupied by a dermoid. In this respect horses and boys differ very markedly, but they agree in the following points : although a dermoid may be 2 J 546 TUMOURS OF THE TESTIS attached to, or incorporated with, either a right or a left testis in fairly equal proportions, an example of bilateral testicular dermoid has yet to be recorded. The frequent presence of hyalin cartilage in malignant tumours of the testis has attracted the attention of many writers. In some cases cartilage forms the chief portion of the tumour and its metastases ; several investigators have endeavoured to determine the nature of the chondrification. In consequence of the frequency with which cartilage is found in these tumours it has been customary to class them as sarcomas. Foulerton has shown that many malignant tumours of Spermatic cord. Epididymis. Testis. Tunica vaginalis. Portion of tmnour within tlie tunica vaginalis. — The tumour. Fig. 288. — Testicle from a child with a tumour growing from the lower pole, which contained muscle -spindles, some of which were transversely striated. {After Naumann.) the testis are in structure and pathologic tendency carcino- mas ; and he points out that Paget's classic specimen when re-examined by Kanthack and Pigg was found to be a carci- noma. He is also of opinion that writers on surgical pathology are in the habit of considering the presence of hyalin cartilage as evidence that the tumour is a sarcoma, and that chondri- fication of tissue in typical cancers has not been sufficiently considered. He has, however, proved in an unequivocal way that hyalin cartilage occurs in association with cancer of the rectum (Fig. 287), and has proved its presence in a lymph- gland infected with carcinoma. Foulerton is also of opinion that many, if not the majority, of malign tumours of the testis SARCOMA 547 are more properly classed with the carcinomas than with the sarcomas ; at the same time he is in agreement with preceding observers that malignant tumours of the testis arise in the hilum of this organ. Tumours containing transversely striated muscle-cells sometimes grow from the testicle (Fig. 288). Sarcomas. — In addition to teratomas arising in the paradidymis, malignant tumours pos'sessing the structure Fig. 289. — Testicle in section. A, Epididymis ; B, sarcomatous tissue ; c, remnant of the body of the oi'gan. From a man aged 28, who had noticed an enlarge- ment of his testicle for eight months. Castration was performed. He died ten months later. and clinical features of sarcomas arise in the body of the testis. Such tumours have oat-shaped cells, or round cells, and some have the characters of lympho-sarcomas. Whatever view pathologists may take of the structural characters of testicular tumours, the surgeon never forgets the grim reality that the majority of these complex growths quickly destroy life (Fig. 289). One of the most prominent clinical features of malignant 548 j iTUMOUBS OF THE TESTIS tumours of the testis is the rapidity and extent of the lymph- gland infection. The great size which the lumbar lymph- glands attain in some patients is truly astonishing. The connective tissue in the hilum of the testis is described as consisting " of fine fasciculi and laminae of areolar tissue, these being covered by and partly composed of flattened epithelioid cells. Between the laminae and fasciculi are large cleft-like spaces, containing lymph and almost everywhere enclosing the basement of the tubules. If these spaces are injected by the puncture method, the injecting fluid flows away by the lymphatics of the spermatic cord " (Quain). This free lymphatic communication of the hilum-territory explains the extreme facility with which the abdominal lymph-system can be infected. Dissemination of testicular sarcoma is some- times brought about by the veins, for it occasionally happens that secondary nodules are found in the skin, lungs and other viscera, nevertheless the prevailing mode of infection is by the lymphatics. This has led several investigators to study the relation of these vessels and their associated nodes to malignant tumours of the testis. Most made a study of this question with the aid of fine injections, and the result of his inquiries is shown in Fig. 290. A study of the relations of these lymph-glands to the cisterna chyli makes it clear how a malignant tumour in the testis leads to enlargements of the left supraclavicular lymph-nodes. Jamieson and Dobson have also made a careful study of this matter (1910). Frequency of malignant tumours of the testis. — During the year 1907 twelve testes were removed in the chief hos- pitals of London for the relief of malignant tumours. The distribution of the cases is as follows : London, 3 ; St. Bar- tholomew's, 2 ; Great Northern, 2 ; Charing Cross, 1 ; Guy's, 1 ; St. George's, 1 ; University College, 1 ; Westminster, 1. At the Middlesex, Cancer, Royal Free, St. Peter's, St. Mary's, and St. Thomas's there was none. Malignant disease in retained testes. — It is commonly believed that retained testes are more liable to malignant disease than those properly lodged in the scrotum. This is one of those articles of surgical faith very difficult to affirm or to deny. In 1909 there were fourteen examples available GANGER OF R-ETAINED TESTIS 549 for study in the metropolitan museums. The youngest patient was 20, the eldest 55. Some of the tumours were large ; for example, Stabb removed one as big as an ostrich's Qgg. In a few of these cases the testis was retained in the abdomen, but in most instances it had entered the inguinal canal When the tumour lies in the abdomen its ovoid shape and lateral position have led the surgeon in at least one instance to regard the tumour as an enlarged kidney. Russell Howard has made a most satisfactory contribution to the question of the increased liability of a retained testis Fig. 290.— Diagram showing the position and relations of the lymph-glands associated with the testicles. The results were obtained by means of fine injections. {After Most.) to become the seat of malignant disease. He states that, in a period of twenty years, fifty-seven consecutive cases of malig- nant disease of the testis were treated in the London Hospital : the malignancy of the tumours was substantiated by a micro- scopic examination. Among this number, in nine cases the testis was retained in the inguinal canal, giving a proportion of nearly 16 per cent. This is certainly a higher proportion than obtains in men with normally descended testes. In addition to malignant disease occurring in retained testes, mention must be made of cancer and sarcoma arising 550 TUMOURS OF THE TESTIS in the ill-developed gonads of individuals with their genitalia so malformed as to come into the class known as pseudo- hermaphrodites. This matter has been investigated by Pick and Zacharias. Thirty-five cases have been reported. It is to me a matter of great interest to learn that the gonads of pseudo-hermaphro- dites are so liable to be the seat of teratomas. In 1887 I expressed the opinion that testicular dermoids probably arise in ovarian tissue entangled in the paradidymis. Clinical features. — The clinical recognition of malignant tumours of the testis is not by any means a simple matter ; it is often impossible to distinguish between a hsematocele and a solid tumour. The points on which it is best to rely are the weight of the tumour and absence of inflammation, syphilis, and translucency. Some sarcomas are intensely hard ; others are soft, and almost fluctuate ; most of them are painless, but a few are the seat of continual pain. Treatment. — A study of the effects of operation for the cure of tumours of the testis is very instructive, as it exhibits malignant disease in some of its worst aspects. Castration, save in very exceptional cases, is one of the safest operations in surgery. In the early stages of sarcoma the diseased testis can be completely removed. Kecurrence in the wound or stump is an unusual event; but dissemination, due to in- fection of the lumbar lymph-glands, destroys more than half the patients within a year of the operation. These glands are in close relation with the inferior vena cava and the ab- dominal aorta, and the intimate association with these blood- vessels was regarded as an obstacle to their removal. In spite of this, cancerous lumbar glands have been extirpated with success. With the hope of improving these results, I introduced (1909) a method of "radical orchidectomy " by which the testicle, the spermatic cord and its veins are completely removed. The free incision necessary for the removal of the spermatic veins allows the surgeon to extirpate the lymph- nodes in the abdomen around the aorta and vena cava. Russell HoAvard has also had a successful casein a boy (1910). Encysted hydrocele of the testis (spermatocele). — The cysts to which the term " encysted hydrocele of the testicle " SPERMATOCELES 651 should be applied arise sometimes in the vasa efferentia ot the testis and sometimes in Kobelt's tubes, and it is a curious fact that these cysts occur in those structures which in the female give rise to parovarian cysts. As encysted hydroceles in the male and parovarian cysts in the female arise in homo- logous organs, these cysts are morphologically homologous. Encysted hydroceles are always closely associated with the testis, and lie outside its tunica vaginalis, but they may pro- ject into the cavity of this sac. Occasionally a hydrocele of the tunica vaginalis is associated with an encysted hydrocele. When an encysted hydrocele is very large it may so over- lap the testis that it is difficult to differentiate between it and a hydrocele of the tunica vaginalis, until actual dissection in the course of an operation shows that the cyst is independent of this tunic. The lining epithelium of such cysts may be of the strati- fied, cubical, columnar, or even of the ciliated variety. The cysts contain fluid, which may be clear, or white like milk, due to the presence of fat ; sometimes spermatozoa are present ; or the fluid may be blood-stained. Cysts containing semen are sometimes called spermatoceles. In size these cysts vary greatly. As a rule they do not exceed the dimen- sions of an egg, and often are much smaller. An encysted hydrocele must not be confounded with a cyst arising in an unobliterated funicular process. In addition to the sessile form of encysted hydrocele of the testis there is a pedunculated variety which is usually described as a supernumerary hydatid of Morgagni. These cysts rarely exceed a cherry in size, and arise in Kobelt's tubules. As a rule only one cyst is present, but two or three are not uncommon. Sometimes they will, like the hydatid of Morgagni, project into the cavity of the tunica vaginalis. These small bodies interest the morphologist ; to the mere surgeon they are only curiosities. Literature. — It is an interesting feature of writings con- cerning dermoids of the testis that the majority of the observations, certainly the best among them, have been the work of French surgical writers. It would seem that the classical monograph of Verneuil gave the subject a French domicile. 552 TUMOUES OF TEE TESTIS Barrington, F. J. F., "A Demoid Cyst of the Testicle."— iarace^^, 1910, ii. 460. Bland-Sutton, J., "An Essay on Dermoids of the Testis."— Arch, of Middx. Ifosp., 1903, i. 19. Bland-Sutton, J., "An Improved Method of removing the Testicle and Spermatic Cord for Malignant Bise-dse."— Lancet, 1909, i. 1406. Bland-Sutton, J., " The Value of the Undescended Testis." — Pract., 1910, p. i. Bland-Sutton, J., Erasmus Wilson Lectures. — Lancet, 1887, i. 256. Chevassu, M., " Tumours du Testicule," Paris, 1906. Cooper, A., " Diseases of the Testes," 1830. Comil and Berger. — Bull, de V Acad, de Med., 1885, xiv. 275. Curling, T. B., " Observations on Cystic Disease of the Testicle." — Med.-Chir. Trans., 1853, xxxvi. 449. Foulerton, A. G. R., " Carcinoma of the Testicle."— ia^cei;, 1905, ii. 1827. Hobday, "The Castration of Cryptorchid Horses,'' London, 1903. Howard, Russell, " Malignant Disease of the Testis."- -Prac?;., 1907, p. 794, Howard, Russell, " A Radical Operation for Malignant Disease of the Testis." — Lancet, 1910, ii. 1406. Hutchinson, J., jun., " Sarcoma of the Testicle." — Trans. Path. Soc, 1889, xl. 193. Jamieson, J. K, and Dobson, J. F., " The Lymphatics of the Testicle." — Lancet, 1910, i. 493. Kanthack and Pigg, S., " Malignant Enchondroma of the Testis." — Trans. Path. Soc, 1827, xlviii. Kuhne, J. E., "Rare Tumour of the TesticlQ.'''— China Med. Journ., 1908, p. 79. Most, " Ueber maligne Hodengeschwiilste und ihre Metastasen." — Virchow's Arch., 1894, cliv. 138. Naumann, Virchow's Arch., ciii. 497. Paget, Sir James, " Account of a Growth of Cartilage in a Testicle and its Lymphatics and other Parts." — Med.-Chrr. Trans., xxxviii. 247. Schlagenhaufer, Fr, " Ueber das vorkommen Chorionepitheliom und trauben molenartigen VVucherungen in Teratomen." — Wien. Mm. Woch., May, 1902, Nos. 22, 25. Verneuil, " Memoire sur I'lnclusion Scrotale et Testiculaire." — Arch. Gen. de Med., 1855, S^e serie, v. 641, and vi. 21, 191, 299. Wilms, " Die teratoiden Geschwiilste des Hodens." — Ziegler's Beit. f. path. Anat., xix. 233. Zacharias, P., " Beitrage zur Kenntniss der Geschwulst biklungen an der keiindrusen von Pseudohermaphroditen." — Arch. f. Gyn., 1909, Ixxxviii. 506. CHAPTER LIV HETEROTOPIC TEETH Among mammals the normal situation for teeth is the mouth (buccal cavity), but under pathologic and teratologic conditions they arise in such unexpected situations as the ovary, testis, rectuui, neck and pharynx, in man, and in connexion with the tympanum of horses (mastoid teeth). Among heterotopic teeth those found in ovarian dermoids (embryomas) are the best known, and have been the subject of several careful investigations. Ovarian teeth. — A large proportion of ovarian dermoids contain teeth. In number they vary greatly. Sometimes only two or three are found ; in others, twenty or more may be counted. Two hundred teeth have been counted in a multi- locular dermoid, but it is an unusual number. The teeth may be embedded in loose, ill-formed bone, or project from a flat osseous plate like nails driven through a piece of thin wood (Fig. 291). Often the roots of ovarian teeth are embedded in soft tissue, or the entire teeth remain hidden in crypts or cysts. When the crown projects boldly the neck of the tooth may be surrounded with pink tissue resembling the gums. Teeth occur more frequently in ovarian dermoids than would be gathered from the current descriptions. Unless the teeth are actually erupted the surgeon may fail to notice them until he makes, or orders to be made, a careful dissection of the dermoid. On several occasions I have astonished my assistants by directing a dermoid in which no dental structures were obvious to be destroyed by prolonged boiling: when the residue was examined many (sometimes twenty or thirty) teeth were found. The teeth are not scattered irregularly through the dermoid unless present in very great number, but are collected in one or more groups ; they vary in shape and resemble 553 554 HETEROTOPIC TEETH incisors, canines, and supernumerary teeth. The root is usually single ; teeth with more than one root, or with a bifurcated root, are very-rare. When the crown is simple the root is long; multicuspidate teeth have short roots. Ovarian teeth are composed of enamel and dentine ; Fig. 291. — Cluster of ovarian teeth embedded in bone. Fig. 292.— Developing ovarian tooth, showing the enamel- organ. From an adult vroman. cementum is by no means constant. The enamel is lodged on the crown in lumps or hummocks, with deep ravines extending to the dentine. The enamel prisms run in all directions. The pulp is very irregular ; some of the teeth, especially those resembling incisors and canines, may lack a central chamber. In a multicuspidate tooth the pulp-chamber OVARIAN TEETH 555 is of fair size. In some the pulp is converted into secondary- dentine (Fig. 293); in others it is full of fat-globules. The presence of nerves in the pulps of ovarian teeth was asserted by Salter, and tissue resembling nerve-fibrils may be detected in pulp suitably prepared. Ovarian teeth develop on the same principle as normal teeth (Figs. 292, 294). Fig. 293. — Microscopic characters of a multicuspidate and bicuspidate ovarian tooth. Fig. 294.— Germ of an ovarian tooth. E, Enamel organ ; p, papilla. For several years I made a series of observations in order to determine if the development and eruption of ovarian teeth is in any way influenced by age, and to ascertain if, like the hair of dermoids, they are shed in old age. The evidence proved that the development of ovarian teeth is uninfluenced by age : for example, a dermoid the size of a tennis-ball, from a girl 6 years of age, contained many teeth, six of which were 556 HETEBOTOPIG TEETH fully erupted ; whereas an ovarian dermoid from a woman in middle life contained germs of teeth in great abundance, but none had reached the stagfe of calcification. Mastoid (tympanic) teeth in horses. — The occurrence Fig. 29.5.— Two dental masses successfully removed from the temporal fossa of the mare represented in Fig. 297. The larger tumour, A, is shown in section to display the enamel strata ; it weighed 175 grm. The smaller body, c, weighed 44 grm. ; it is also shown in section, b. of teeth in the mastoid portion of the temporal bone in horses has been known for upwards of a century, and specimens of these curious teeth exist in many veterinary museums. The number of teeth varies ; as a rule one tooth is present, stuck like a peg in the bone. It is not uncommon to find two, MASTOID TESTS 557 and in rare instances four teeth. Mastoid teeth are very misshapen, and usually of the molar type ; often they are such ill-fornied lumps as to come under the denomination odontomes; indeed, Broca included these bodies among his odontomes heterotopiques (Fig. 295). Mastoid teeth possess Fig. 296. — The tympanic region of a horse's skull with a cluster of teeth. the three familiar dental tissues — enamel, dentine, and cementum. A careful examination of the very few available specimens in which the skull has been preserved with the mastoid teeth in position shows that they arise in relation with the tympanum, and especially with that part of it known as the attic. This is true of a specimen in the museum of the Royal Veterinary College, London, in which a solitary tooth 558 HETEBOTOPIG TEETH stands out from tlie remains of its bony capsule, the roots of the tooth being lodged in the tympanic attic. Owing to the kindness of Professor Devvar I was able to study carefully a skull with the teeth in position (Fig. 296). In this specimen the teeth are not lodged in sockets, but encysted by an incomplete bony capsule, in the mastoid portion of the temporal bone, especially in that part imme- diately overlapped by the squamosal. The cluster of teeth has markedly compressed the external auditory meatus. The tumour has deformed the interior of the cranium, and an uncovered portion of tooth projects into the cerebellar fossa. During life it was probably excluded by the dura mater. It is impossible to determine accurately the number of separate dental bodies in this specimen without destroying it, but I feel sure there are at least four separate pieces. There is no satisfactory explanation available as to the origin of these teeth. There is, one would certainly imagine, some morphological reason for their localization in the temporal bone, and, as the drawings show, they have peculiarities in shape and size which should serve for their ready recognition, and enable us at once to distinguish them from testicular or ovarian teeth. Before it is possible to make any decisive statement in regard to the nature of the mastoid teeth of horses, it is very desirable to obtain facts concerning their anatomical relation- ship with the soft parts. Mastoid teeth are troublesome to horses, and give rise to some interesting clinical conditions. The horse is usually brought to the veterinary surgeon on account of a swelling, but more frequently a sinus, near the base ■ of the auricle. When a probe is passed along the sinus it comes in contact with a tooth. The recorded cases of this disease fail to make it clear whether the sinus is congenital or is a consequence of suppuration as the tooth develops. Heusinger, in an admirable paper on cervical fistulse, regards them as per- sistent branchial fistulse, and states that they are more frequent in carriage-horses (Luxuspferde) than in draught- horses, as the secretion from the sinus soils the surrounding skin and attracts the attention of the grooms. This sinus is very constantly associated with mastoid teeth in foals as GEBVIGAL TEETH 559 well as in adult and aged horses. The veterinarian usually treats these cases in a summary manner, for he enlarges the sinus and, ascertaining the exact position of the tooth, extracts it by forceps or by a chisel and mallet ; he then stuffs the cavity with antiseptic gauze, and encourages it to become obliterated by granulation. A study of the character and position of these teeth shows that their removal is some- times attended with difficulty, certainly with grave danger to the horse, and occasionally their extraction is impracticable. Fig. 297. — Head of a van mare witli a sinus leading to a mastoid tooth. The drooping lip shows that there was paralysis of the facial nerve. Cases are known in which horses have died from septic meningitis, the result of suppuration around the teeth. Cervical teeth in sheep. — Sheep are liable to a peculiar anomaly in the immediate neighbourhood of the ear, which consists of a fistula opening near its base ; but its skin edge is invariably surmounted by an incisor tooth. The first impression is that the opening represents a persistent branchial fistula, but in man teeth are not associated with these fistulse. Congenital cervical fistulte in sheep have received careful attention, and these investigations show 560 HETEROTOPIC TEETH that the abnormal orifice is an accessary mouth. In an example which came under my notice (Fig. 298) the tooth, which had the characters of a temporary incisor, is lodged in a bony pedicle surrounded by mucous membrane of the same features as the gums, and the cutaneous recess in which it is accommodated presents, in the aspect which is in contact with the tooth, a number of processes resembling the papillae Auditory meatus Accessary mouth Fiff. 298. Head of a sheep with a cervical teratoma. In the lower figure the teratoma is shown of natural size. on the sheep's lips, and the arrangement of the wool on the outer surface of this accessary lip is identical with that covering its normal hp. This specimen by itself is some- what puzzling, but a wider survey of the question adds a special interest to it. Gurlt had the opportunity of studying several examples which enabled him to prove conclusively the nature of this condition, and in one of his specimens two temporary incisors CERVICAL TEETH 661 were lodofed in a miniature but unmistakable mandible, and associated with a tongue of corresponding size. The fistulous track communicated with the pharynx. When the animal drank, some of the fluid escaped through the fistula. Without entering too fully into the details of this matter, the revelation afforded by a thorough anatomical study of the specimens amounts to this : — The cervical teeth and the associated structures are the remnants of an attached or 'parasitic foetus, and the cuta- neous opening represents its mouth. It is rare for an animal with one of these accessary mouths Fig. '299. — Head of a cross-bred Devon cow with a teratoma attached to its throat. A cane passed through the fistula a entered the pharynx of the cow. to come under the notice of a trained observer, so I gladly avail myself of the notes taken by Mr. Wilson, a veterinary surgeon, concerning a lamb. Some few days after the lamb was born, the shepherd noticed that the wool on the right shoulder was saturated with milk. He carefully watched the lamb suckling, and on close examination discovered a slit behind the mandible, through which the milk issued. He drew his master's attention to this, and the latter, to his astonish- ment, found a rudimentary tongue and jaw covered with a lip: naturally, he kept the animal alive out of curiosity. When the lamb was weaned and turned out on pasture land there was always a food-stained condition of the wool around the 2 K 562 HETEROTOPIC TEETH opening ; the animal appeared to maintain a decent con- dition. When turned out in the winter it lost the use of its front legs, and was taken to the farm buildings, kept warm, and hand-fed. At this stage Mr. Wilson saw it, and found a pharyngeal fissure three inches in length, the tongue freely movable and working in harmony with the normal tongue. Broca, " Traite des Tumeurs," 1869, ii. 3G9. Dewar, Journ. of Comj}. FatU., xvi. 127. Goubaux, Recueil de Med. Vet., 1854, xxxi. Heusinger, Deutsche ZeUschr.f. Thiermedicin, 1876, ii. Magitot, "Traite d'Anomalies de Systeme Dentaire," 1877, PI. ix. Mettams, Veterinarian, Ixxii. 309. Roll, ZeitsoTir. d. K.K. Gesellscfiaft der Aerzte in Wien, Marz, 1851, Heft 3 S. xliii. Walley, Journ. of Comp. Path., ii. 152. For the cervical teeth of sheep see — Berger-Perriere, Recueil de Med. Vet., xii. 586. Giirlt, " Tliierische Missgeburten," Berlin, 1877, Taf. xv. Wilson, W. T., Private Letter. GROUP Vn. CYSTS CHAPTER LV RETENTION-CYSTS Cysts, or cystomas, result from the abnormal dilatation of pre-existing tubules or cavities. In the simplest form they consist of a wall usually composed of fibrous tissue, but it not infrequently contains muscle-fibre. The cyst-contents may be mucus, bile, saliva, urine, etc., according to the nature of the organ with which the cyst is associated. Cysts may be arranged in three groups, thus: 1. Retention-cysts. 2. Tubulo-cysts. 3. Hydroceles. There are some conditions often classed as cysts which in this work will be arranged as a sub-group under the title Pseudo-Cysts, and will embrace — i. Diverticula, ii. Bursse. iii. Neural cysts. In this and the next chapter Ave shall deal with reten- tion-cysts. When the duct of a gland becomes obstructed, the fluid, hindered from escaping, accumulates in the ducts and acini and dilates them. If the hindrance to the free flow of the secretion is maintained, or often repeated, the glandular tissue becomes impaired, then atrophies, and finally the gland and its duct become converted into a fluid-containing sac or cyst. It is generally believed that when the duct of a gland is completely obstructed the conversion of the parts into a cyst is a passive process ; but occasion will be taken in the course of this section to show that that is not the case. When an excretory duct is so completely obstructed that no secretion escapes, then the gland rapidly atrophies. 563 564 GY8T8 Retention-cysts are caused by obstruction to the free flow of secretion, or temporary arrests of the flow frequently recurring. The best examples of cysts arising in this way are those due to dilatation of the pelvis and infundibula of the kidney — a condition known by the terra hydronephrosis. The purest forms of retention-cysts arise in connexion with hollow organs, the inner walls of which are provided with glands. The vermiform appendix is a case in point. This tubular structure is richly provided with glands. Occasionally the communication of the appendix with the csecum is obstructed, and, the glands continuing to secrete, the accumulating fluid distends the appendix into a sausage- shaped cyst. The uterus is another example. After a difficult labour the walls .of the cervical canal are not infrequently damaged, and in the process of repair the canal may become obstructed. This leads to retention of the products secreted by the uterine glands, and the uterus will attain such proportions as to cause the enlargement to be attributed to pregnancy; the condition is known as hydrometra. It is occasionally seen in old women, but more commonly in mammals noimally furnished with bicornate uteri, such as ewes, cows, mares, and sows. It may be unilateral or bilateral. When occurring in mammals in which the uterus has long cornua — e.g. the cat, bitch, hare — the distended cornua are apt to be confounded with Fallopian tubes. One or both cornua of a human bicornate uterus may be affected. The danger of retention of this kind is due not so much to the size of the cyst as to the great risk that ensues when large collections of retained secretions are invaded by putre- factive organisms. The cysts in such an event become con- verted into abscesses, and the life of the individual is greatly imperilled. These changes in retention-cysts are indicated by special names — as pyometra, pyonephrosis, etc. Cysts of the liver. — All cysts, in the pathologic limita- tion of the term, arise in pre-existing epithelium-lined spaces and ducts : even ducts and canals of microscopic proportions are often the source of cysts of such dimensions that they cause so much inconvenience and distress as to necessitate GYSTS OF THE LIVER 566 surgical treatment. This is illustrated by the liver, an organ thoroughly permeated by minute passages — the bile-canals. Two forms of cysts are found in the liver, arising from its canals and ducts — namely, multiple cysts and solitary cysts. Fig. 300. — Liver shown in section. The spaces on the cut surface are dilated bile-canals. From a woman 46 years of age. (Museicm of the Royal College of Surgeons, London.) 1. Multiple cysts of the liver. — This variety has long been recognized by pathologists under the term general cystic disease of the liver. In typical examples the liver is converted into a huge honeycomb-like mass (Fig. 300). The cavities 566 CYSTS vary greatly in size — some are as small as grape- seed, others may exceed a ripe cherry in size. The cysts may project on the surface of the liver, but though this organ may be enormously enlarged and Aveigh thirty-five pounds, yet its shape is preserved. The smaller cysts are lined with epithe- lium. This is best seen in specimens with the cystic change in an early stage, when the dilated canals look like sharp, definite punctures in the liver substance. As the cysts increase in size and number, the hepatic tissue is encroached upon, and appears as narrow bridges between large tracts of honeycomb, but by degrees these become broken up by absorption, and then the remnants of the normal hepatic tissue are seen as islands on the cut surface of the liver. The microscopic characters of the cysts when examined in the early stages prove that they arise in the bile-canals, but no investigator has succeeded in ascertaining the cause of this disease, or in associating it with obstruction to the escape of bile. The most remarkable circumstance connected with this disease of the liver is its occasional association with general cystic disease of the kidneys {see p. 590). This general cystic disease of the liver is productive of great enlargement of the organ, but is painless, causes no jaundice, presents no diagnostic features, and comes invariably as a post-mortem surprise. This curious disease has attracted the attention of several pathologists, including Virchow, Kokitansky, Bristowe, Still, Shattock, and Rolleston. Blackburn, in a careful and critical paper, has reviewed the various theories relating to this disease and collected the literature. 2. Solitary (non-]:)arasitic) cyst of the liver. — This is a rare condition, and the general character of such a cyst may be inferred from the specimen represented in Fig. 301. In nearly all the recorded cases the cysts grow from the free margin of the liver and possess thin walls which are trans- lucent and have no communication with the gall-bladder. The peritoneal investment and the capsule of the liver are directly continuous with the cyst-wall. On microscopic ex- amination of a large solitary cyst, which I enucleated from the liver of a woman 75 years of age, the cyst-wall at the point where it joins the liver exhibited small loculi lined with GY8T8 OF THE LIVER 567 epithelium ; ducts could also be detected lined with cubic cells. The cyst-wall consisted of fibrous tissue, and its inner surface presented spaces covered with flattened epithelium. In some parts of the cyst- wall, liver substance was detected. In such cysts the fluid may be straw-coloured bile, or blood. The solitary cyst of the liver probably arises from the dilatation and fusion of bile-ducts, and, althouo'h it is difiicult BO^t Fig. 301. — Cyst (non-parasitic) growing from the free border of the liver. Obtained post-mortem from a woman 38 years of age. {Museum of the Royal College of Surgeons.) to explain its origin, attention may be drawn to the following points. The recorded examples occurred in women. It is noteworthy that the liver of many women presents along its free border a variable strip of thin atrophied tissue, which appears almost white in contrast with the dark hue of the normal liver. This atrophy of the free border of the liver is attributed, and I think correctly, to the pressure of stays ; whether this be true or not, it was in this pale, thin strip of 568 CYSTS liver that the sohtary non-parasitic cyst arose in my patient, and it was due to the dilatation of the bile-ducts in this tissue ; the dilated ducts subsequently fused to form larger spaces, much in the same way that cystic spaces arise in a cavernous nsevus from the fusion of adjacent blood-vessels composing the primary nsevus. Few special treatises mention the solitary cyst of the Fig. 302. — Gall-bladder distended -with mucus secondary to obsti-uction of the cystic duct with gall-stones. The triangular piece of liver attached to it was removed with the gall-bladder. liver, but a sufficient number of examples have been recorded to prove that it is a clinical entity and may require surgical treatment. In a patient under my care the cyst contained two pints of straw-coloured fluid and simulated a mesenteric cyst. One physician who saw the patient regarded the swelling as an ovarian cyst. In regard to treatment, two methods have been adopted. The common plan consists in ojiening the cyst, evacuating GALL-BLADDEB 569 its contents, and then draining it. This is tedious : in my case I succeeded in enucleating the cyst- wall, with the best consequences. It is worth notice that multiple cysts of the liver admit of no treatment, and, as far as I know, do not admit of diagnosis ; the solitary (non-parasitic) cyst is a clinical puzzle, but is amenable to surgery. The gall-bladder. — This structure illustrates very well the mode of formation of retention-cysts. The gall-bladder consists of three coats, of which the middle one contains un- striped muscle-fibre ; the inner one is mucous membrane beset with mucous glands, its epithelium being directly continuous with that lining the hepatic ducts on the one hand and with the epithelium covering the duodenum on the other. The outer coat is derived from the peritoneum and subserous tissue. Bile from the hepatic ducts is conveyed into the gall- bladder by way of the cystic duct, and when it escapes from the gall-bladder it again traverses the cystic duct and passes along the common bile-duct to the duodenum. The common duct just as it enters the wall of the intestine receives the duct of the pancreas. The point of junction is indicated by a slight recess known as the ampulla or diverticulum of Yater. The peculiar arrangement of the ducts leading to and from the gall-bladder renders it specially liable to have its com- munications interfered with. Obstruction may occur in the cystic duct (Fig. 302), in the common duct, in the ampulla, or in the wall of the duodenum. The obstruction may be due to impacted gall-stones, a pancreatic concretion in the diverticulum, tumours of the pancreas, duodenum, primary cancer of the common bile-duct, etc. When the common duct is obstructed by gall-stones, the gall-bladder usually atrophies in consequence of cholecystitis. In obstruction due to cancer of the head of the pancreas the gall-bladder becomes, as a rule, greatly distended with bile, When the cystic duct is obstructed, and no bile finds its way into the gall-bladder, the latter may become so distended with mucoid fluid and attain such large proportions as to be mistaken for an ovarian cyst. A gall-bladder distended in this way is really a viucocele, and the consistence of the mucus varies greatly. 570 CYSTS Ranula. — This term is probably one of the oldest in surgery, and its etymology is not very obvious. Until recentl}^ it was applied to all cysts in the floor of the mouth, and as cysts in this situation are of various kinds and arise from different structures, it naturally followed that the term gradually came to possess a merely topographical signifi- cance. There is at the present time a strong tendency to restrict the name ranula to cysts arising in connexion with the ducts of the three sets of salivary glands opening into the mouth, and to designate them as submaxillary, sublingual, or parotid ranulse, according to the gland affected. If sur- ofeons w^ould use the term in this definite sense much un- necessary discussion would be saved. Ranulse are common in connexion with the submaxillary and sublingual glands. The cysts are, as a rule, thin- walled, and lie in the furrow between the gum and the tongue, and bulge upwards into the floor o± the mouth. When large they cause a prominence in the submaxillary triangle. The cyst may be filled with saliva. Sometimes it contains mucus and a yellow substance resembling the yolk of an egg. Occasionally the obstruction is caused by a calculus impacted in the orifice of the duct, but cases come under observation in which the duct is not completely obstructed, yet the fluid is retained. Observation teaches that w^hen the main excretory duct of the submaxillary gland is blocked by a calculus, inflammatory (infective) changes follow in the gland, which subsequently produce hardening (sclerosis) of its tissue. Cystic changes are exceptional, and there is, in all probability, a pathological cause apart from mere obstruc- tion concerned in their production. Parotid ranulse are rare in the human subject, but they have been observed in calves, oxen, and horses. Much needless discussion has taken place in regard to the sources of ranulse, because the various writers seem to forget that in addition to sahvary glands there are mucous glands, and one of variable size, near the tip of the tongue, known as Nuhn's gland. Any of these may dilate into a cyst. Still further to complicate the diagnosis, dermoid cysts not infre- quently arise in the floor of the mouth, near the frsenum ol the tongue, or deeply in its substance. It has also been urged, PANGREATIG GYSTS 571 as an objection to the view that ranulse arise in the ducts of the salivary glands, that the fluid they contain is not always saliva. This is very weak argument. Many hydronephrotic cysts contain fluid which it would be difficult to regard as urine, and an obstructed gall-bladder is sometimes filled with fluid that does not possess a single attribute of bile. So a cyst arising in connexion with a salivary gland will sometimes contain fluid that fails to furnish the characteristic reactions of saliva. Pancreatic cysts. — It has long been known that the duct of the pancreas is liable to become dilated, and as the condition is analogous to the distension of the ducts of the buccal salivary glands, dilatation of the pancreatic duct (canal of Wirsung) is sometimes referred to as a " pan- creatic ranula." Virchow recognized two varieties of pancreatic ranula. In one variety the canal is dilated irregularly throughout its whole extent, so that it assumes the appearance of a chaplet of cysts ; in the other the duct is dilated immediately behind its terminal orifice. Such cysts, he writes, may attain the size of a fist, and are consecutive to cicatricial contractions and compression by tumours. The cysts are not filled simply with pancreatic secretion, for when they attain a certain size they will be found to contain mucoid material, products of haemorrhages, and, not rarely, calculi. Judging from what is known of retention-cysts in general, it would, as a matter of simple inference, be thought that pancreatic ranulte arise from partial obstruction to the pancreatic duct, either from impaction of a pancreatic calculus in the terminal segment of the duct, a gall-stone lodged at the duodenal orifice, or a tumour arising in connexion with the ducts or tissues in the immediate neighbourhood. This, however, does not appear to be the case, for pancreatic cysts have been observed and no obstruction has been detected. Besides this, the duct of the pancreas has been found completely obstructed by a ■calculus, and the gland, instead of being converted into a cyst, has atrophied, its secreting elements being largely replaced by fibrous tissue. Experimental evidence also supports this conclusion, for it has been demonstrated that when the pancreatic duct is 572 CYSTS occluded during life by a ligature, tlie gland does not become cystic, but atrophies. Thus experimenfal and clinical evi- dence indicates that pancreatic cysts are the result of patho- logical changes which may, or may not, be associated with obstruction of the duct. A great deal of attention has, during the past ten years, been devoted to pancreatic cysts in their clinical as well as their pathological aspect, and certainly the evidence indicates that other causes than obstruction, partial or complete, are responsible for their production. Cysts described as pancreatic sometimes attain very large proportions, and examples have been reported with a capacity of two gallons or more. These very big cysts form smooth globular swellings in the upper part of the belly. They lie behind the peritoneum, and, of course, have the stomach and transverse colon in front ; when very large these cysts will extend some distance below the transverse colon. The fluid contained in large pancreatic cysts is usually turbid. Sometimes it is white or even opalescent, occa- sionally it is clear, and in some cysts it will have a brown or even a green tint. The specific gravity varies between 1010 and 1020, and there is a small trace of albumin. Mucin is often present, also tyrosin and blood-pigment, and traces of urea have been detected. The fluid is sometimes capable of emulsifying fats. The modes by which very large pancreatic cysts arise are not by any means clear, but it is important to bear in mind that there is, in a very significant proportion of cases, a definite histor}^ of antecedent injury. This fact gives colour to the suggestion that some of the cysts are due primarily to laceration of the pancreas and subsequent extravasation of its secretion behind the peritoneum. Another very important feature of these cysts is the liability to haemorrhage, and this may take place so abundantly into the cyst as to jeopardize the life of the patient; indeed, in some cases it has been fatal. Pancreatic cysts occur at almost all periods of life. Ex- amples have been reported as early as the eighth year of life and as late as the seventy- third. They appear to be more frequent in men than in women. Pancreatic cysts attributed to injury have followed a DAGUYOPS 578 variety of accidents, siich as a fall from a great height, caus- ing abdominal pain ; a crush of the abdomen between the buffers of railway waggons ; falls from a horse, or from a vehicle ; kicks from men, and in several cases from horses. ' Jordan Lloyd has attempted to show that the large pan- creatic cysts that follow injury to the abdomen are really collections of fluid in the cavity of the lesser omentum, and when the fluid has the property of rapidly converting starch into sugar it may be assumed that the pancreas has been injured. He also points out that the characteristic feature of so-called pancreatic cysts — viz. a swelling occupying the epigastric, umbilical, and left hypochondriac regions — is pre- cisely that which would result from distension of the lesser bag of the peritoneum. It is probable that some cases of supposed pancreatic cysts were really effusions into the lesser bag of the peritoneum, for undoubted examples of distension of this cavity with fluid have been observed, dissected, and described. The whole subject of so-called pancreatic cysts has been ably handled and the literature collected by Leith. Dacryops. — This term is applied to cysts occurring in the upper eyelid ; they are due to distension of the ducts of the lachrymal gland. They appear, as a rule, in the upper and outer part of the eyelid, the cyst extending beneath the border of the orbit towards the lachrymal gland. The cyst enlarges when the patient weeps. Dacryops may arise in two ways — either as a consequence of a wound or abscess of the lid, or as a congenital defect. As a rule, they are of traumatic origin. The condition is one of extreme rarity. Hulke, in an interesting paper on this subject, states his belief that these cysts were first accurately described by Dr. J. A. Schmidt in 1803, and that Beer (1817) mentions that he had six cases of this kind, which he describes under the name " dacryops," previously applied to them by Schmidt. When these cysts are opened through the skin a fistula is sure to be the result. The same thing often happens when the cysts have a traumatic origin. The condition is then termed dacryops fistidosus. Cysts of the hyaloid canal. — This tiny relic, which sometimes persists in the vitreous after the disappearance of the central hyaloid artery, may occasionally dilate and 574 OYSTS form a cyst large enough to be visible on ophthalmoscopic examination. Blackburn, " Cystic Disease of the Liver and Kidney." — Trans. Path. Soc, vol. Iv. 203 ; with a complete list of references. Bland-Sutton, J., " On Solitary (non-parasitic) Cysts of the Liver." — Brit. Med- Journ., 1905, ii. 1167. Doran, A., Med.-Chir. Trans., 1904, Ixxxvii. 1, with literature. Hulke, J. W., " Dacryops ; Dacryops Fistulosus Palpebrse Superioris." — Boy. Lond. OiMhal. Hosp. Rejrts., 18.57-59, i. 285. Leith, R. F. C, " Euptnres of the Pancreas : their relations to pancreatic cysts ; with some remarks upon treatment." — Edin. Med. Journ., 1895, xli. 423. Lloyd, Jordan, "Injury to the Pancreas: a cause of effusions into the lesser peritoneal cavity." — Brit. Med. Journ., 1892, ii. 1051. Morton, C. A., Lancet, 1905, ii. 1395. RoUeston, H. D., " Diseases of the Liver," 1905. Sharkey, S. J., " Simple Cyst in connection with the Liver." — Trans. Path. Soc., 1882, xxxiii. 168. Sharkey, S. J., and Glutton, H. H., "Case of Pancreatic Cyst; successful removal." — St. Tliomas's Hasp. Repts., 1893, xxi. 271. Still, G. F., " Congenital Cystic Liver and Cystic Kidney." — Trans. Path. So ., 1898, xlix. 155. CHAPTER LVI RETENTION-CYSTS (Concluded) H Y DUONEPHROSIS The secretion (urine) of the kidneys is conclucted into the bladder by means of two ducts (the ureters) ; from the bladder the urine is discharged at intervals through the urethra. When from various causes the urine is hindered from escaping freely, either from the bladder, or from the ureters into the bladder, it accumulates in the ureters and dilates them ; the pressure of the fluid then acts upon the pelves of the kidneys, and if maintained causes the renal pelves to be dilated into large sacs, converts the infundibula into large tubes, and finally induces atrophy of the renal tissue until the kidneys are converted into multilocular sacs. To a kidney altered in this way the term hydronephrosis (Rayer, 1839) is applied. Hydronephrosis arises from a variety of causes, and the condition of the ureter associated with it depends on the cause and situation of the obstruction. It is also important to bear in mind that the largest examples of hydronephrosis are produced by partial obstruction to the flow of urine or by frequently recurring attacks of complete obstruction. It is also a curious fact that in many of the largest examples of hydronephrosis it is difficult to demonstrate the cause. Hydronephrosis may be bilateral or unilateral. When the obstruction is at the neck of the bladder or in the urethra it will be bilateral. The chief causes of bilateral hydronephrosis are — Calculus in the urethra or in the bladder (Fig. 303). Stricture of the urethra. Tumours of the prostate gland ; especially an enlarged middle lobe. Tumours and cysts of the pelvic organs ; especially impacted uterine and cervix fibroids. 575 576 CYSTS In bilateral hydroneplirosis secondary to obstruction at the neck of the bladder, an interesting change may sometimes be observed at the vesical orifices of the ureters. Normall}^ these openings scarcely admit a fine probe, but under the conditions Fig. 303. — Hydronepkrosis secondary to a large calculus in the bladder ; two fragments of calculus occupy the prostatic portion of the urethra. The left kidney was in a similar condition. The patient, a man 26 years of age, died with complete suppression of urine. {Jfiiseion, Middlesex Hospital.') (j ncd. size. ) just mentioned they will assume a circular form, and be large enough to admit the tip of the little finger, so that fluid injected into the bladder through the urethra will enter the ureters and gain the dilated pelves of the kidneys. This HYDBONEPHBOSIS 577 condition is particularly apt to supervene upon oft-repeated attacks of retention of urine secondary to pressure on the urethra exercised by a uterus occupied by fibroids, especially Fig. 304. — The urinary organs with the right adrenal of a new -torn child. those which grow in the cervix and become impacted immediately before the incidence of a menstrual period. Antenatal hydronephrosis. — A very large number of ex- amples of hydronephrosis have been carefully examined and recorded in children at birth, and in some of the specimens the distension of the pelves of the kidneys has been so great as to 2l 578 CYSTS obstruct delivery and entail embryotomy in order to allow of the extraction of the foetus. In many of the records the reporters state that they were unable to find anj'thing to explain the condition ; in some of the more recent cases, however, the cause of the obstruction has been determined. The chief of these causes are : — ■ Imperforate urethra (Figs. 314, 31G). Imperforate hymen (Fig. 317). Torsion of the penis (Fig. 305). Antenatal hydronephrosis is a subject of great interest, because it serves to show that the kidneys are functional in the late stages of fcetal life, and supports the view that the bath of amnionic fluid (or hydrosphere) in which the foetus floats represents, at any rate in part, foetal urine. It also throws some light on cases of advanced hydronephrosis some- times met with in infants and young children, and for which no adequate cause had previously been forthcoming. The urinary organs represented in Fig. 304 were obtained from an infant which survived its birth a few days. The right kidney only was present ; it occupied its normal position in the loin. Its infundibula, pelvis, and ureter were widely dilated, and at the point where the ureter opened into the bladder there was a small circular diaphragm-like valve, but this structure offered no obstruction to the flow of fluid from the ureter into the bladder when tested after death. The bladder presented only one ureteral orifice, and its walls were thinner than usual. The penis, urethra, and tes- ticles were normal, and the left adrenal occupied its usual position. No traces of the left renal artery, vein, or ureter were found. The anus was normal. In this case dissection failed to bring to light anything to account for the distension of the excretory ducts of the kidney, but it clearly indicated that mechanical obstruction of some kind interfered with the flow of urine through the vesical orifice of the ureter. Torsion of the penis. — It is an undoubted fact that torsion of the penis and bilateral hydronephrosis are some- times associated, and it is possible that in some cases of congenital double hydronephrosis in which there was great dilatation of the ureters and in which careful dissection of H YDR ONE PER OS IS 579 the parts failed to detect any organic cause, twisting of the penis may have been overlooked. A baby three months old had his penis directed laterally ; the organ was brought into a natural position, but on being released it at once resumed its abnormal deflection to the left (Fig. 305). This penis, as is the case with twisted penes in general, was unusually large. There was also a groove on the under-surface of the glans indicating a minimum degree of hypospadias; the frtenum was absent. At the angle of torsion there was a sac-like pouch of skin. The penis was probably distorted in this way by the pressure Fig. 30,5.— Twisted peuis. The small figure shows the groove on the glans and the absence of the frsenum. {Xat. size.) of the thighs whilst in the uterus. It is possible that the penis may be nipped between the thighs and may obstruct the urethra and lead to hydronephrosis without its being actually twisted. Unilateral hydronephrosis has many causes: — Retention of a calculus in the ureter. Tumour of the bladder implicating the vesical orifice of the ureter. Calculus lodged in the pelvis of the kidney and block- ing the orifice of the ureter (Fig. 311). Partial rotation of the kidney kinking the ureter. Tumours and cysts of the pelvic organs pressing on the ureter. 580 CF^ST^S' Cicatrix of the ureter due to injury. Inadequacy of the ureter (Fig. 309). It has been suggested that the ureter is occasionally ob- structed by branches of the renal artery taking an abnormal course. I have made a careful study of cases supposed to rig. 306. — Kidney iii the conditiou known as hj'di'oueplu'osis, showing the way in which the renal vessels are stretched across the sac and interfere with the ureter. demonstrate this, and have been convinced that the unusual relation of the vessels is often due to the dilatation of the renal pelvis, and that the constricting effects supposed to be exercised by the artery are the direct outcome of the increase in the size of the pelvis (Fig. 306). Among uncommon varieties of unilateral hydronephrosis may be mentioned sacculation of one half of a horseshoe HYDRONEPHEOS'IS 581 kidney, and the rarer anomaly in which a kidney is furnished with two ureters, one of which becomes obstructed and leads to dilatation, of that portion of the renal pelvis connected with it, and corresponding atrophy of that part of the renal cortex which drains into it. Intermitting hydronephrosis. — When a hydronephrotic kidney is of large size it can be perceived clinically as a definite tumour. It occasionally happens that patients come under observation with a swelling in the loin which can be readily perceived at one examination but not at another, or it obviously diminishes in bulk without completely vanishing. In some of these cases the patients are able to state definitely that, coincidently with the diminution in the volume of the tumour, there has been a sudden increase in the quantity of the urine voided. The urine in some instances has been found to contain traces of blood and mucus. To hydrone- phrosis of this kind the term interrtiitting is applied. It must be borne in mind that there may be difficulty in some cases in deciding clinically between a very large hydro- nephrotic cyst and an ovarian or parovarian cyst, and it is well established that cysts of the ovary and parovarium sometimes rupture, and the fluid, escaping into the perito- neum, is absorbed, and rapidly excreted by the kidneys. Thus, jjrofuse diuresis following ui^on the sudden disap- jjearance of an abdominal tumour is as characteristic of rupt^ure of an ovarian cyst as of an intermitting renal cyst. There can be little doubt that nearly all hydronephroses intermit, but the term intermitting hydronephrosis is reserved for those examples in which there is great diminution, and in some instances temporary disappearance, of the swelling. Exceptionally, both kidneys when hydronephrotic may intermit alternately. Of this rare form I have had one case under my care; as the diagnosis was somewhat obscure, cceliotomy was performed. In the course of the operation the phenomenon of intermission was actually observed. The hydronephrosis diminished in size, and the bladder slowly filled. Intermitting hydronephrosis is also associated with the remarkable anomaly known as inadequate ureter. It is a startling fact that many of the largest specimens of hydronephrosis are those in which no obstruction could 582 CYSTS be demonstrated, and the histories of the patients fail to throw any hght on the cause (Fig. 307). The most remark- able example of this is the case of Mary Nix, who died at Hampton Poyle, near Oxford, at the age of 23. She had a hydronephrosis containing fluid to the amount of thirty gallons, wine measure. The dissection of the body was conducted by Samuel Glass with "some learned gentlemen of Fig. 307. — Unilateral (intermitting) hydronephrosis (without obvious cause). The ureter, at the point where it left the renal sinus, had a diameter of 8 cm. Nothing was found to account for the the university, condition. Now that we know many cases of dilated ureter and sacculated kidney have an antenatal cause, it is very prob- able that many large hydronephroses of inexplicable origin in the adult began while the individual tenanted the uterus. Inadequate ureter, — It is well recognized that the junc- tion of the renal pelvis with the ureter proper is indicated by B YDEONEPHBOSIS 583 a marked narrowing of the lumen of the duct, which may be conveniently called the " ureteral strait." It occasionally happens that the ureter from this point downwards is very markedly narrow, and is some- times even less than one-fourth its proper dimensions (Fig. 309). Abnormally narrow ureters of this kind may be convenient- ly termed " inadequate." Five examples of this condition have come under my observation, and in each instance it was asso- ciated with intermitting hydro- nephrosis. It is an extraordinary fact in some of these cases that the urine will collect and form a sac holding two, three, or even four litres, and produce very great pain ; suddenly, and without any warning, the blockade will be raised, and the urine will pass into the bladder and be voided, and the laro-e cystic swelling subside in a night. This excessive narrow- ness of the ureter is most prob- ably a congenital defect. The insidious way in Avhich the gradual dilatation of the renal pelvis, infundibula, and calyces destroys a kidney is very extraordinary. When hydro- nephrosis is unilateral it rarely betrays itself until the tumoiu' is very large ; often the only trouble it causes is increased frequency of micturition. When the hydronephrosis , -1 ^ 1 ,1 . ^ „ Fig. 308.— Hydi'o-ureter without ob- IS bilateral the signs are often vious cause. From an old mau. 584 CYSTS in abeyance until tlie amount of renal capital is reduced to the minimum amiount capable of meeting the ordinary de- mands of the individual ; directly there is an extra call, then the small balance of available renal tissue becomes alarmingly manifest, and the patient dies. A rare combination of inadequate ureter and sacculated Fig. 309.— Dilated renal pelvis associated with an inadequate ureter. kidney is to find a sacculus stuffed with a multitude of small calculi. The kidney in Fig. 310 is of this character. I removed it from a man in the Middlesex Hospital in 1904. The calculi are in the museum of the Royal College of Surgeons, which contains a similar specimen, not only as regards the large number of calculi and their shape, but also in the fact that the kidney which contained them was " hydronephrotic and the ureter narrowed." The specimen was obtained after death from a man aged 19 years. PYONEPHROSIS 585 Pyonephrosis. — Although a hydronephrosis continues its course in secret, it is ahiiost certain to be made manifest when it suppurates, and my observations among the quick and the dead have taught me that this is one of the greatest dangers to which an individual with unilateral hydronephrosis is liable (Fig. 311). Fig. 310. — Sacculated kidney with au inadequate ua-eter ; the largest sacculus con- tained more than 40,000 calculi, which were iridescent and resembled little balls of burnished gold. Removed from a man 38 years of age. It is necessary to draw a distinction between pyone- phrosis and suppurating hydronephrosis. In the case of a pyonephrosis the lesion is inflammatory from the outset, whether it starts primarily in the kidney or spreads to this gland from the bladder, ureter, or elsewhere. In some cases of suppurating hydronephrosis under my own care, I have been satisfied that the colon was the source 586 CYSTS of infection, and the pus contained Bacillus coli coinmunis. The intimate relations existing between the kidney and colon, more especially when the former is hydronephrotic, make one wonder that fistulous communication between these viscera is not a frequent complication. Congenital cystic kidney.— This term is applied to a very Fig. 311. — Pyonephrosis in section; the pelvis at its junction with the ureter is partially blocked by a calculus. {Miiseii/ji of the Ro>jaI College of Surgeons.) characteristic disease of the kidneys. In tj^pical examples these organs are converted into cystic masses, so that they exhibit a sponge-like appearance on section. The cysts vary greatly in size ; some are as small as rape-seeds, others as large as cherries ; they rarely exceed these dimensions. Some of the cysts project from the surface of the kidney, but, though interfering with the smoothness of the gland, they do not distort it. The cortical and medullary portions of such CONGENITAL CYSTIC KIDNEY 587 kidneys are indistinguishably blended, but here and there tracts of cortical tissue may be detected among the cysts (Fig. 312). In the early stages the cyst-walls have a membrana propria, and are lined with tesselated epithelium. In ad- vanced stages of the disease and in the large cysts the epi- thelium disappears. A striking feature of these kidneys is the narrowness of the ureters, and yet in all the cases which have come under my observation these ducts have been Fig. 312. — Cougeiiital cystic kidney in section. {Xat. size.) {Museum of the Hoijal College of Surgeons.') pervious throughout. The arteries supplying kidneys changed in this way are smaller than normal. A congenitally cystic kidney sometimes attains an enormous size, so large indeed as seriously to impede labour, and in many cases embryotomy has been necessary in order to enable delivery to be effected. In a large proportion of instances in which the foetus comes away without difficulty it is still-born and often malformed; such conditions as anencephalia, club-foot, and spina bifida are often associated 588 GTSTS with congenital cystic disease of tlie kidneys. Minor degrees of the affection are not incomjDatible with life, and several in- stances are known in vdiich such kidneys have been found in adult individuals. Although this condition of kidney is very common and specimens illustrating it exist in many pathological museums, Ave know very little concerning the early stages. I have ex- amined a well-marked example in a foetus of the sixth month, Fig. 313. — Congenital cystic kidney ; early stage. {Shattock.) and Shattock observed one at the fourth month. The earliest stage has been observed by Shattock (Fig. 318), and a careful examination of the minute structure of the cyst, as well as a comparison of the histology of the cyst with that of the meso- nephros (Wolffian body), induced him to think it probable that these kidneys consisted of a combination of mesonephros and metanephros. Virchow regarded the cj^sts as dilatations of the uriniefrous tubules in consequence of the absence of a renal pelvis. It is, however, a curious fact that " congenital cystic kidney " occasionally occurs in association with im- CONGENITAL CYSTIC KIDNEY 589 perforate urethra. A very remarkable case came under my notice in which a child born at full term, but with great difficulty on account of the large size of its belly, was found to have an imperforate urethra, a large dilated left ureter, and a hydronephrotic left kidney. The opposite kidney was a typical example of the congenital cystic kidney (Fig. 314). Adrenal. Bladder. Fig. 314. — Urinary organs of a foetus. The urethra is imperforate, the bladder hyper- trophied, the left ureter dilated, and the kidney hydronephrotic. The right kidney is a congeries of cysts (congenital cystic kidney). This combination of the two forms of hydronephrotic and con- genital cystic kidney in the same individual, associated with imperforate urethra, supports Yirchow's view that the cysts are due to ectasia of uriniferous tubules. The large number of specimens of congenital cystic kidney preserved in museums indicates that the condition, if advanced at the time of birth, is incompatible with life. It 590 CYSTS is, however, quite certain tliat a precisely similar change is met with in adults, and, what is also remarkable, it is very frequently associated with a similar change in the liver (see p. 566). The available facts indicate that a moderate amount of cystic change in the kidneys is not incompatible Avith life, but as the disease advances the secreting tissue of the organs is slowly but surely destroyed, and in due course uraemia supervenes and the patient dies. Fig. 315. — Localized cystic disease of the kidney. {After Edmunds.) There is reason to believe that this cystic change may be limited to part of a kidne}^. Edmunds described a speci- men (Fig. 315) which he removed from a girl of 18 years, in which " an encapsuled tumour " projected into one of the calyces of the kidney. It had a diameter of 6 cm., and was composed of a " congeries of cysts " lined with cubical epi- thelium. The large cyst connected Avith the side of the bladder CONGENITAL CYSTIC KIDNEY 591 represented in Fig. 314 had a capacity of 300 c.c. ; it is a dilated ureter. Such a condition is often mistaken for a bifid bladder. Assuming that the congenital cystic kidney is due to pressure leading to dilatation of the uriniferous tubules, we are met by a difficulty in dealing with the specimen, Fig. 314 : Fig. 316. — Fcetus of the fourth mouth, with imperforate urethra and a large dis- tended bladder. The kidneys were cystic, as in Fig. 314. (Shattock.) the same cause has produced hydronephrosis in one kidney and general cystic disease in its fellow. I think the difference may be, in part perhaps, explained by the period of intra- uterine life at which the obstruction manifests itself Shattock described a foetus of the fourth month with an imperforate urethra; this fcetus had a very large dilated 592 GY8T8 bladder (Fig. 316), and both kidneys were typical examples of general cystic disease. The museum of St. Bartholomew's Hospital contains the reproductive organs of a child born at full time : the hymen was imperforate and the bladder greatly distended ; the vagina was converted into a large cyst containing mucus (Fig. 317). The pressure of the distended vagina had com- pressed the urethra, causing retention of urine which over- filled the bladder, and dilated the renal pelvis, producing the condition known as sacculated kidney. A comparison of these Fig. 317.— Kidney, uterus, vagina, and bladder of a new-bom child, shown in section. The distension of the vagina and uterus is due to an imperforate hjTnen. The ureter is widely dilated and the kidney sacculated. {Museum of St. Barthohmciv'' s Sospifal.) specimens seems to indicate that the dilatation of the urini- ferous tubules which results in general cystic disease of the kidneys is due to some cause acting very early in embryonic life. When obstruction to the outlet of urine occurs in CONGENITAL GYSTIG KIDNEY 593 the later stages of intra-uterine life, dilatation of the renal pelvis and its recesses (hydronephrosis) is the more frequent consequence. It is, however, a point of some importance to realize that there are cases in which the kidneys, though the seat of general cystic disease, are capable of performing their function, and the individuals attain adult life. In such cases the cysts of the kidneys increase in size, and the organs attain the pro- portions of full-grown pumpkins. Eventually the secreting substance of the kidneys is destroyed and the patient slowly dies from ursemia. It is important to realize this condition of kidney, because in several instances surgeons have re- moved organs thus enlarged : such interference has not been to the advantage of the patients. The museum of the Royal Colleo'e of Surgeons, London, contains an excellent series of specimens illustrating general cystic disease of the kidney in man and other mammals. Bland-Sutton, J., " A Kidney which contained more than 40,000 Calculi." — Lancet, 1905, i. 125. Edmunds, W., " Cystic Adenoma of Kidney." — Trans. Path. Soc, 1892, xliii. 89. Glass, S., "An Uncommon Dropsy from the want of a Kidney; and descrip- tion of a large Saccus that contained the Water, sent to Dr. Mead." — PJiil. Trans., 1747, xliv. 337. Pye-Smith, P. H., " Cystic Disease of the Liver and both Kidneys." — Trans. Path. Soc, 1881, xxxii. 112. Shattock, S. G., " Imperforate Urethra in a Foetus of about the fourth month." — Trans. Path. Soc, 1888, xxxix. 185. Virchow, R., " Congenitale Nierenwassersucht." — Gesamvite Aihandl. z, wissen- schaft MecUcin, 1856, p. 839. 2m CHAPTER LVII TUBULO-CYSTS The human body contains certain tubes which, so far as is known, serve no useful purpose in the adult, and may be called in consequence functionless ducts. Some of these — e.o-. the vitello-intestinal duct and the urachus — were probably useful to the embryo ; others, like the parovarium and Gartner's duct, are serviceable in the male, as they act as conduits to the testis. Functionless ducts must not be con- founded with obsolete canals : these serve no useful purpose in man, but were, in all probability, functional in the ances- tors of existing vertebrates (Chap. xlvi.). Both sets of canals are of interest to the pathologist, as they are the source of cysts which are not only inconvenient to the individual but actually dangerous to life. The genus tubulo-cysts includes the seven following species : (1) Cysts of the vitello-intestinal duct ; (2) cysts of the urachus ; (3) paroophoronic cysts ; (4} parovarian cysts (p. 514) ; (5) cystic disease (adenoma) of the testis (p. 539) ; (6) cysts of Gartner's duct (p. 518) ; (7) cysts of Miiller's duct. Cysts of the vitello-intestinal duct. — It is not un- common to find connected with the umbilicus of babes and young children small tumours varying in size from a pea to a cherry. These tumours are bright-red, soft and velvety to the touch, and are, as a rule, connected with the navel by slender pedicles, and in appearance resemble red currants; occasionally they are sessile. These tumours are composed of unstriped-muscle fibre, mucous membrane, Lieberkubn's follicles, and columnar epi- thelium, collected into a mass. Typical cases have been carefully described by many observers. In rarer cases the umbilicus is occupied by a cyst, which may project externally or internally. Such a cyst is lined 594 GYSTS OF TEE VITELLINE DUCT 595 Lung diverticulum. Stomach. with mucous membrane furnished with villi, columnar epithe- lium, and follicles. A cyst of this character is easily con- founded with the sac of an umbilical hernia. The histology and position of pedunculated tumours and sessile cysts at the navel indicate the structure from which they arise — viz. a remnant of the vitello-intestinal duct which, in the embryo, traverses this part of the abdominal wall (Fig. 318). In transverse sections of the umbilical cord, close to the belly wall of the embryo at the fifth month, the vitello- intestinal duct can often be detected, with its lumen lined with subcolumnar epithelium. It is also well known that the duct, instead of shrivel- ling, sometimes grows j^ari ■passu with the gut with which it is connected, and acquires a lumen almost equal to that of the ileum. Instead of per- sisting from the gut to the navel, the duct may atrophy, leaving a small portion at- tached to the intestine or to the abdominal wall. Such remnants may develop into cysts the walls of which are identical in structure with those of the small intestine. A much rarer variety of cvst arising in a remnant of the vitello-intestinal duct is due to the distension of that portion of the duct which is connected with the ileum. In recently hatched chicks the intestinal attachment of the duct is often indicated by a nipple-like process on the free border of the gut. This is hollow, but does not communicate with the lumen of the ileum. As a rule it atrophies completely. It may, however, grow and form a large cyst. The museum of the Royal College of Surgeons contains a piece of intestine from an emu chick with a large cyst suspended from it by means of a narrow and acutely torsioned pedicle. This cyst, Fis Vitello-intestinal duct. , 318. — Diagram of the alimentary canal of the embryo, showing the position of the yolk-sac. 596 TUBULO-CTSTS in all probability, originated in a persistent portion of the vitello-intestinal duct. Cysts of like proportions and of identical origin have been recorded in the human subject. One of the. best-known cases was reported by Roth. Occasionally a persistent vitello-intestinal duct will remain open at the umbilicus and discharge fseces. • Such cases have been successfully dealt with by surgeons. Sherren removed a Meckel's diverticulum (Fig. 319) from a man ao'ed 38 : it contained concretions composed of cholesterin, calcium oxalate, and bile-pigment. A valve existed at the junction of the diverticulum with the ileum. The operation was undertaken for acute colic. A diverticulum is present in about 2 per cent, of the population. It is a very troublesome structure: Gray, in a careful paper, states that 150 cases of intestinal obstruction caused by it were reported between 1893 and 1906. " From 10 to 20 years appears to be the age at which these accidents are most common. It is also liable to acute inflammation, or diverticulitis, which is as dangerous as acute ap- pendicitis." There are few structures in our bodies more capable of exciting philo- sophical speculation than the yolk-sac and its duct. This organ may in man and all the higher mammals be regarded as vestigial, for its duties have been in part abrogated by the allantois, but more completely by the placenta. In the human embryo it is the function of the allantois to convey the blood-vessels which it receives from the developing aorta and distribute them to those chorionic villi destined to form the foBtal portion of the placenta. In some sharks the yolk-sac is covered with vascular Fig. 319. — A Meckers diverticulum containing concretions ; it commu- nicated "with the lumen of the ileum by a narrow oijening protected by a valve. {After Sherren.) IMPERFORATE ILEUM 597 villous tufts wliicli fit into depressions of the oviduct. Even in some mammals — e.g. guinea-pigs — the yolk-sac enters into vascular connexion with the uterine mucous membrane. There are abundant and good reasons for Balfour's conclusions that placental mammals are descendants of forms the em- br3^os of which had large yolk-sacs; but the yolk became reduced in quantity owing to the nutriment the embryo received from the maternal tissues by means of the vascular connexion of the yolk-sac with the uterine wall. Subse- Fig. 320.— Septate ileum. {Museum of the Middlesex Rospital.) quently the function of the yolk-sac became limited by the allantois and the gradual evolution of the placenta, and finally, so far as man is concerned, abolished. Thus in man it is vestigial, and, like such structures in general, is liable to many vagaries. There is good reason to believe that the vitello-intestinal duct, besides being a source of cysts, is also responsible for the curious defect in t,he ileum to which I have applied the name imperforate ileum. It occasionally happens that the lumen of the ileum is interrupted by a perforated diaphragm (Fig. 320). To such a condition the term septate ileum is 598 TVBULO-GYSTS applicable. When such a diaphragm is present its situation is so]iietimes indicated by a marked constriction of the gut. In other specimens a more or less perfect valve of this kind is associated with a persistent duct. In such cases the duct opens into the ileum on the distal side of the valve. In other instances the ileum becomes greatly dilated near its middle, and the walls are much hypertrophied ; to this . succeeds a narrow isthmus which opens into a normal segment of ileum. Lastly, in the comjDlete form the ileum is interrupted (Fig. 321). Proximal segment of ileum. Free edge of mesentery. Distal segment of ileum. Pig. 321. — Imperforate ileum. {Museum of the Jliddlesex JlosjjitaJ.) These curious defects are attributable to the influence of the vitello-intestinal duct, because they always occur in that portion of the ileum to which the duct, when persistent, is attached — that is, they do not occur within 30 cm. of the ileo-csecal valve, and are rarely found at a greater distance than 1 metre from the caecum. The most reliable evidence for associating these defects with the duct of the yolk-sac is that furnished by specimens in which a persistent duct and a valve coexist. In my early observations I had regarded imperforate ileum as depending CYSTS OF TEE UBAGHUS 599 upon the influence of the vitello-intestinal duct, and sub- sequent observations put the speculation on a sound basis. The specimens which demonstrate these views are preserved in the museum of the Middlesex Hospital. Cysts of the urachus. — The urinary bladder of man, in common with that of other mammals, presents at its apex an impervious cord that passes to the umbilicus. This cord, known as the urachus, is traversed at birth by a narrow canal lined with epithelium directly continuous with that of the bladder. The urinary bladder with the urachus is the persistent portion of the allantois, the organ which in the early embryo conveys blood-vessels from the aorta to the developing placenta. In the adult the urachus lies in the subperitoneal tissue exactly in the middle line of the anterior abdominal wall, between the summit of the bladder and the umbilicus. When the urachus becomes dilated it forms a cyst lying outside the peritoneum and in close relation with the bladder. Instead of a portion of the allantois narrowing to form a urachus, the whole of its intra-abdominal portion may dilate and form a large urinary bladder. Several cases are known in which the umbilical end of the urachus has remained patent, so that urine was voided at this spot. A urinary calculus has been extracted from such a persistent urachus. Cj^sts of the dimensions of a cherry are not uncommon in the urachus, especially near the summit of the bladder; sometimes a number of small dilatations occur, causing the urachus to assume a moniliform appearance. In rare cases the urachus may dilate into a cj'st as large as a distended bladder. The walls of such cysts are composed of unstriped-muscle tissue. The surgeon must be on his guard not to confound a sacculus at the apex of the bladder, or extending into the suspensory ligament, with a cyst arising in the urachus. Lawson Tait drew attention to the probable origin of some forms of extraperitoneal cysts in the urachus, and the whole matter has been subjected to a very critical and painstaking analysis by Doran. 600 TUBUL0-GYST8 Allantoic (urachus) cysts not only occur in man, but I have observed them also in the pig, horse, ox, and mole. Ci/sts of AliUler's ducts. — In many vertebrata the eggs, after their escape from the ovary, are conveyed to the exterior by means of a muscular conduit known as the oviduct. The general disposition of these ducts, for there are usually two, may be gathered from an exam- ination of a female frog or toad. The ducts extend from the cloaca posteriorly to the roots of the lungs anteriorly ; they are supported on the dorsal wall of the abdomen by means of a delicate fold of peri- toneum, and each duct communicates with the peritoneal cavity by a dilated orifice known as the infundibulum. In the breeding season the ducts become greatly enlarged and convoluted, resembling coils of small intestine. Normally, oviducts are present in the female only. It is, however, remarkable that the embryos of those forms in which the sexes are distinct in the adult condition have the rudiments of the sexual organs peculiar to the male and female ; they are hermaphrodite. As develop- ment continues, one set of organs usually attains a functional condition ; the other atrophies more or less completely. The distinguishing features of the ihternal sexual organs of a female frog are two ovaries and two oviducts. In the male the oviducts are usually absent. It is, however, an interesting fact that in many male frogs the oviducts may be detected as thin, delicate threads ascending in the peritoneum from the structures called vesiculae seminales to the roots of the lungs. Sometimes the ducts are of large size, almost equal to the oviducts in the female. Persistent MLillerian ducts are more common in male toads than in frogs. Often they are associated with the malformation of the genital gland known as an ova-testis ; but they are fairly frequent even when the genital gland is a typical testis. No one can doubt that an oviduct in a male frog or toad is functionless, and it is not uncommon to meet with small dilatations or cysts lying in the track of, and arising from, the functionless oviducts. Persistent Miiller's ducts are by no means confined to batrachians, but they have been observed in fish, lizards, stallions, birds, and men. Good examples of cysts arising in functionless ducts are sometimes met with in birds. In birds, as in frogs and toads, the eggs are con- veyed to the exterior by means of an oviduct, but in the case of birds the duct is functional on the left side only. Each chick has two oviducts, but the right ovary and duct, from some unexplained cause, atrophies, leaving, as a rule, a small, narrow tubule surmounted by a lobule of fat. This remnant of the right duct is very apt to dilate and form a cyst. When the stump of the duct is longer than usual it will sometimes become unequally dilated and form a chaplet of cysts. Bland-Sutton, J., " Abstract of the Erasmus Wilson Lectures on the Value of Comparative Pathology to Philosophical Surgery " : Lecture l.Srit. Med. Jour)i., 1891, i. 342. 11EFEBENGE8 601 Doran, A., " Urachal Cysts." -Proc. Roy. Soc. Med., Surgical Section, 1909, ii. 198. Gray, H. T., "Some Cases in which Meckel's Diverticulum was present." — Brit. Med. Journ., 1907, ii. 823. Paget, T., "A Case in which the Urachus remained open, and a ring-shaped Calculus, formed upon a Hair in the Bladder, was extracted through the Umbilicus." Communicated by William Bowman, F.R.S. — Med.-Chir. Trans., 1850, xxxiii. 293. Sherren, J., " Meckel's Diverticulum containing' Calculi and producing Colic." — ProG. Roy. Sog. Med., Clinical Section, 1910, iii. 11. CHAPTER LVIII HYDROCELE The name hydrocele is applied to several different kinds of cystic tumours, and as the term is so deeply rooted in surgical literature it would be very inconvenient to attempt to dis- card it. In this work it will be restricted to cysts clue to an excessive accumulation of fluid in a diverticulum or pouch of the peritoneum,, such as — 1, hydrocele of the tunica vaginalis; 2, hydrocele of the canal of Nuck ; 3, ovarian hydrocele ; 4, omental hydrocele. 1. Hydrocele of the tunica vaginalis. — Each testicle is preceded in its descent by a diverticulum of the parietal peritoneum, which enters the scrotum by way of the inguinal canal. As the testicle descends behind this diverticulum, or funicular pouch, as it is termed, it invaginates the membrane in such a way as to invest the anterior two-thirds of its surface with a double layer of peritoneum. When the testicle first gains the scrotum the funicular pouch is in free communica- tion with the general peritoneal cavity. It is a remarkable fact that in almost every mammal, male and female, save man, this relation of the funicular pouch to the peritoneal cavity persists throughout life. The only exceptions which have come under my notice occurred in a chimpanzee and a gorilla. In exceptional instances this communication persists throughout life even in man. Normally the peritoneum be- comes adherent immediately above the testis, this adhesion dividing the pouch into two parts ; the portion in relation with the testis persists throughout life as the tunica vaginalis, whilst that above the testis usually undergoes obliteration in the course of the early months of infant life. Normally, then, the only portion of the funicular pouch which persists throughout life is that in immediate relation 602 HYDROGELES 603 with the testis, and when it becomes distended with fluid it is termed Itydrocele of the tunica vaginalis. When contain- ing blood it is called Jicumatocele of the tunica vaginalis. Should the whole of the funicular pouch persist and be- come occupied by fluid, it is called a congenital hydrocele. Frequently the tunica vaginalis is formed as usual, but the portion intervening between it and the internal abdominal ring persists and may become distended with fluid. This is known as funicular hydrocele; it is often called encysted hydrocele of the cord. Hydrocele of the tunica vaginalis appears in two forms, acute and chronic. Acute hydrocele is due to inflammatory effusion into the sac, either the result, of injury or secondary to acute orchitis. This is the rarer form, and, as a rule, the fluid is absorbed and the parts return to their normal condition as the inflammatory trouble that caused it subsides. Exceptionally a hydrocele appearing in this way persists. The common form of hydrocele is a passive effusion into the tunica vaginalis, usually appearing about the middle period of life, and in many cases without any exciting cause, either local or constitutional. It is very common in men who have lived in the tropics. Hydrocele is met with in extreme old age, and is occasionally bilateral. The amount of fluid in hydroceles varies greatly ; in some it amounts to one or two ounces, whilst in others it mea- sures a pint or more. The fluid is limpid, of a straw colour, with a specific gravity of about 1015. It contains a large amount of albumin and the substance known as fibrinogen. When allowed to stand after withdrawal it spontaneously coagulates. When the fluid is removed by tapping it usually quickly reaccumulates, so that the amount of fluid furnished by a large hydrocele in the course of a few years is often considerable. A native of the Gold Coast had a hydrocele of the tunica vaginalis which contained fifteen pints of fluid (Horn). The presence of a large quantity of fluid in the tunica vaginalis leads to changes not only in the membrane itself but also in the testicle, for this gland, pressed upon by the fluid, will in course of time atrophy. In most specimens the testis is situated in the lower and back part of the sac, as in 604 CYSTS Fig. 322. In those cases in wliich the testis is inverted the hydrocele projects posteriorly, and the testis lies in front and at the upper part of the sac. In addition to atrophy of the testis, there may be great thickening of its tunica albuginea, a condition termed peri- orchitis, which may mask the diminution in the size of its secreting tissue, and is by no means infrequent in old hydro- celes, especially those which have been repeatedly tapped. Covering of the cord Cremaster muscle. Tunica vaginalis. Fig. 322. — Hydrocele of the tunica vaginalis testis. This thickening, or sclerosis, manifested by the immediate covering of the testis, is often seen in the tunica vaginalis throughout its whole extent, and in some cases this mem- brane may be as thick and almost as hard as pasteboard. The hardness of these thick sacs is sometimes increased by calcareous matter. When such sacs are dissected out they are not unlike a coco-nut in shape, size, and consistence. Second- ary changes of this kind are attributed to repeated attacks of inflammation set up by tapping : in some cases bands of adhesions or broad septa form, and produce a loculated cj^st. HYDB0GELE8 605 In other cases suppuration ensues, which may lead to serious consequences. Occasionally, loose bodies are found in the sac of the tunica vaginalis, often associated with, but sometimes independent of, hydroceles. Some are no larger than the head of a pin, others attain the dimensions of a cherry. The larger examples consist of dense, structureless laminae. Rupture of a hydrocele. — This is a rare accident, and is the result of slight injury or a nmscular strain ; in some cases it appears to have happened spontaneously. Hastings has collected forty cases, and the records show that the accident occurs mainly in chronic hydroceles and is associated with degenerative changes in their tunics. In two cases under my care the patients complained of pain, followed by oedema, and, some hours later, ecchymosis of the scrotum due to extravasation of the fluid into its loose tissues. One of the men burst his hydrocele when stooping to pick up a sixpenny-piece ; the other crushed his scrotum against the corner of the table when playing bilhards. The variety known as congenital hydrocele is due to the persistence of the funicular pouch throughout its whole extent. In this form we meet with two conditions : the sac may retain its connexion with the general peritoneal cavity, or it may be occluded at the internal abdominal ring. When the orifice of the sac is not occluded, the fluid which accumulates in the sac gravitates into it from the peritoneal cavity during the day ; but during the night, when the body has been in a recumbent position for a prolonged period, the fluid returns wholly or in part to the abdomen, so that in the morning the scrotal swelling will be found greatly diminished, if not entirely gone. As the day goes on the fluid will slowly re- accumulate in the tunica vaginalis. Such alteration in size of the swelling is characteristic of this variety of hydrocele • but it is sometimes simulated by, and mistaken for, an ingui- nal hernia. A child under my observation had tuberculous peritonitis and a congenital hydrocele : the peritoneal fluid infected the hydrocele-sac with tubercle. I removed the testis, its cord and coverings, and found tubercle bacilli in the lesions. When the funicular pouch is shut off at the inguinal canal and becomes distended with fluid, it is sometimes diflicult to 606 . GYST8 distinguish it, except by dissection, from a hydrocele of the tunica vaginalis. Congenital hydrocele is most commonly met with in children, and is very rare after the fifteenth year. An accumu- lation of fluid is not uncommon in the funicular pouch of infants, and it often disappears spontaneously. Funicular hydrocele is another variety, frequently referred to as encysted hydrocele of the cord. It is due to effusion of fluid into that portion of the funicular pouch which intervenes between the tunica vaginalis and the internal abdominal rinsT, and which, under normal conditions, suffers obliteration. This form of hydrocele is very frequent in infants, and presents itself as an ovoid tumour lying between the testis and the inguinal canal. Although it possesses very characteristic features, tliis variety of hydrocele is frequently confounded with hernia of the intestines into the funicular pouch. Funicular hydroceles occasionally occur in j^oung adults. It should be borne in mind that an inguinal hernia may be associated with a hydrocele, and it happens occasionally that the neck of a hernial sac becomes so narrowed that gut and omentum no longer pass through it. A pouch of this kind would, if distended with fluid, simulate a hydrocele of the tunica vaginalis ; it is known as hydrocele of a hernial sac. According to Horrocks, the large scrotal cyst which troubled Gibbon, the historian, was an irreducible hernia with a large quantity of fluid in the sac. 2. Hydrocele of the canal of Nuck. — In female fcetusea a diverticulum of the parietal peritoneum descends into the inguinal canal, and is in all respects identical with the funicular pouch in the male ; it is known as the canal of Nuck. Usually this pouch becomes obliterated, but it is by no means rare to find it patent in young women. Occasionally the canal becomes distended with fluid and forms a cyst occupying the inguinal canal, and is then termed a hj'drocele of the canal of Nuck. Treatment of hydrocele. — The routine practice of treating a hydrocele of the tunica vaginalis is to draw off the fluid by means of a narrow trocar and cannula. The cyst almost in- variably refills, necessitating repeated tapping. The simplest method is to open the sac, and, after inverting the tunica HYDROGELE'S 607 vaginalis, return it with the testis into the scrotum. This is also the most appropriate method of treatment for rupture of the tunica vaginalis testis. Nash has shown that rupture of the tunica vaginalis does not often cure the hydrocele. Hydrocele of the canal of Nuck should be dissected out and the neck of the sac ligatured. The same method answers well for hydrocele of a hernial sac, whether inguinal or femoral. 3. Ovarian hydrocele. — The ovaries in rats and mice are contained within a serous sac derived from the peritoneum. The abdominal ostium of the Fallopian tube communicates with the ovarian sac ; hence, when the ova escape from the Ovarian hydrocele in a rat. (Nat. size.) ovary they enter the Fallopian .tube and gain the uterus without entering the general peritoneal cavity, as is the case with the human ovum. This serous sac investment of the ovary recalls the tunica vaginalis of the testicle, and, like it, the ovarian sac is liable to become distended Avith serous fluid, a condition to which I have applied the name ovarian hydrocele. Cysts of this kind in rats may attain a large size, and their general features are well illustrated in Fig. 323- The Fallopian tube in the rat is coiled up between the cornu of the uterus and the ovarian sac, but when the sac be- comes distended it uncoils the tube and stretches it around the circumference of the cyst ; the tubal ostium opens on the inner wall of the hydrocele, and the adjacent section 608 CYSTS of the tube is, as a rule, dilated. The ovary, when the cyst is small, projects into the cysts, but in very large hydroceles it atrojohies from pressure. As the ovarian sac is in communica- tion with the uterine cornu it sometimes becomes implicated in septic conditions of the uterus, and the sac may be found distended with pus. No other mammal normally possesses such a complete ovarian sac as do rats and mice, but many have a pouch that communicates with the general peritoneal cavity by a small aperture ; in others the pouch has a narrow slit ; whilst in women the ovar}^, in its virgin condition, lies in a shallow recess. Notwithstanding the fact that the mouth of the ovarian pouch is in women very wide, there is good reason to believe that its edges may unite when the pouch is abnormally deep and convert it into a closed sac, which subsequently becomes a hydrocele. Ovarian hydroceles occur in the human female, and sometimes attain a large size. They present the following anatomical features : — The sac projects from, and is intimately connected with, the posterior layer of the broad ligament. In small hydro- celes the ovary projects into the cavity of the cyst, but in large examples it is atrophied. The Fallopian tube lies on the crown of the cyst, its outer half is dilated and tortuous ; the ostium opens into the hydrocele by a large circular or elliptical ajDerture. Ridges of mucous membrane issue from the interior of the tube and pass on to the walls of the hydrocele in a radiating fashion. AVhen the specimens are examined in a fresh state it is not rare to find the aperture fringed with tubal fimbria. The general appearance of a typical ovarian hj^drocele suggests " a retort with a convoluted delivery-tube " (Grifiith). Besides finding ovarian hydroceles in rats and women, I have detected one in a guinea-pig, and Schneidemlihl has observed one in a mare. The cysts liable to be confounded with ovarian hydroceles are parovarian cysts, small paroophoronic cysts, and large hydrosalpinges. A parovarian or paroophoronic cystis distin- guished from a hydrocele of the ovary by the fact that the Fallopian tube is stretched across the cyst but does not communicate with its cavity. HYDROCELES 609 In the case of a large hydrosalpinx the ampulla is often so flexed on the tube as to produce a retort-shaped cyst ; but there are no fringes or ridges of the mucous membrane at the orifice of communication, and the ovary lies free of the cyst- wall and is often lodged in the flexure of the tube. Ovarian hydroceles must not be confounded with tubo- ovarian cysts and abscesses the result of salpingitis. 4. Omental hydrocele. — Under normal conditions the lesser cavity of the peritoneum extends into the great omen- tum ; occasionally this space becomes distended with fluid, and its communication with the upper area of the lesser cavity becoming shut ofl", the omental space is isolated and converted into a cyst. Similar cysts arise in the transverse mesocolon, and I have encountered them in the mesocsecum and mesosigmoid. Omental hydroceles are sometimes so big as to simulate ovarian tumours. Chyle-cysts. — This is perhaps the best place to mention a rare but interesting lesion known as chyle-cyst of the mesentery. The sac of the cyst appears to be formed of the separated layers of the mesentery, the interspace being occupied by fluid identical in its physical and chemical characters with chyle. In their anatomical features these cysts are similar to omental hydroceles, and, like them, are occasionally big enough clinically to simulate ovarian cysts (Rasch, Bramann, Mendes de Leon, and Fetherston). There is a variety of cyst containing chyle which is met with in infants and children. Such cysts are closely con- nected with the mesenteric border of the intestine and push their way between the layers of the mesentery. There is reason to believe that they arise as abstrictions of the in- testines during fcetal life. This variety has been particularly studied by Eve, Fawcett, and Dowd. These cysts are im- portant, for they have caused fatal intestinal obstruction. Bland-Sutton, J., " Inguinal Hernia in Monkeys." — Trans. Path. Soc, 1888, xxxix. 453. Bramann, F., " Ueber Chyluscysten des Mesenteriums." — Arch.f.Miti. Chir., 1887, XXXV. 204. Doran, A., " Cyst of the Great Omentum."— Tra?*.'. Obstet. Soc, Zond., 1881-82, xxiii. 164. Dowd, C. N., "Mesenteric Cysts." — Aoin. of Surg., 1900, xxxii. 51. 2 N eiO CYSTS Eve, F. S., "On Mesenteric Cysts ; with two cases in young children subjected to operation." — Med.-Chir. Trans., 1898, Ixxxi. 51. Fawcett, J., "'Chyle' Cyst of Mesentery; Intestinal Obstruction." — Trans. Path. Soc, 1902, liii. 406. Fetherston, R. H., "A Case of Chyle Cyst of the Mesentery." — Austral. Med. Journ., 1890, xii. 475. Frankl,* " Einiges iiber die Involution des Scheidenfortsazes und die Hullen des Hodens." — Arcli.f. Anat. und Entwich., 1895, p. 339. Hastings, S., "Rupture of the Tunica Vaginalis in Hydroceles." — Froc. Roy. Soc. Med., Surgical Section, 1910, iii. 165. Horn, A. E., "A Pifteen-Pint Hydrocele."— ^T-iiJ. Med. Journ., 1907, ii. 143. Horrocks, W., "Medical Notes on the Life of Edmund Gibbon, the Historian." —Lancet, 1901, i. 1356. Mendes de Leon, "A Case of Chyle Cyst." — Amer. Journ. Ohstet., 1891, xxiv. 168. Moynihan, B. G. A., "Mesenteric Cysts." — Ann. of Surg., 1897. Nash, W. G., " Repeated Rupture of the Tunica Vaginalis." — Brit. Med. Journ., 1907, ii. 1065. Rasch, A., " A Case of Large Chylous Cyst of the Mesentery." — Trans. Ohstet. Soc, 1889-90, xxxi. 311. Robinson, A,, " On the Peritoneal Relations of the Mammalian Ovary." — Joiirn. of Anat. and Phys., sxi. 169. * Frankl regards the xiew of the relations of the testicle to its membranes which is set out on page 602 as erroneous. His investigations show that the body of the testis in the embryo projects into the peritoneal cavity, and its intraperi- toneal surface, hke that of the ovary, is covered with involuting germ- epithelium. The body of the descended testis projects into the funicular jDOuch, but it is not covered by the serous membrane. The only part of the testicle invested with peritoneum is the epididymis. The body of the testis has a peritoneal envelope, but not a peritoneal covering. CHAPTER LIX PSEUDO-CYSTS DIVERTICULA The term diverticulum is used to denote hernia or protrusion of the Hning membrane of a cavity through a defective spot in its walls. Such protrusions occur in connexion with the oesophagus and intestines, the bladder, and the trachea ; also in relation with joints and tendon-sheaths, forming synovial cysts and ganglia; and in blood-vessels, forming sacculated aneurysms and varices. Intestinal diverticula. — These are hernial protrusions of the mucous membrane of the bowel through interspaces in the muscular coat. Structurally they consist of mucous membrane with a covering of peritoneum. Sometimes a few strands of muscle-fibre can be detected stretched across the pouch. Frequently diverticula occur in multiples ; as many as two hundred have been found in one case. These pouches occur in all parts of the intestine, but are most frequent in the colon, and especially about the sigmoid flexure. In the small intestine they usually occur along the line of the attachment of the mesentery. In the colon they are found about the attachment of the appendices epiploicse, and may even project into them. In dimensions diverticula vary greatly — some are as small as peas, others as large as oranges. When the pouches are numerous, as a rule they are small ; when few in number, or solitary, they may be large. Intestinal diverticula are common in old persons, but they rarely lead to serious consequences. Some writers describe diverticula of the intestines as con- sisting of two varieties, true and false. According to this arrangement a persistent vitello- intestinal duct would be called a true diverticulum. 611 612 PSBUD0-CT8T8 Vesical diverticula. — Hernial protrusions of tlie mucous membrane of the bladder between the fasciculi of the muscular coat are of frequent occurrence. The cause of the protrusion is impediment to the free flow of urine : the obstruction may- be seated in the urethra or at the neck of the bladder. Under such conditions there may be several diverticula; the bladder is then said to be sacculated. Sometimes there is only one saccule, and this may attain a large size. Vesical diverticula usually communicate with the cavity of the bladder by large orifices. A sacculus extending into the suspensory ligament of the bladder must not be confounded with a urachus cyst. Sacculated bladders, apart from the cause that produces the sacculi, do not often give rise to trouble. Calculi are sometimes found within them, and in cases where the outflow of urine is seriously obstructed the walls of a sacculus will sometimes yield and allow the urine to extravasate into the surrounding loose connective tissue. As impediments to the free escape of urine from the bladder occur more frequently in men than in women, it naturally follows that sacculated bladders are more common in men. Nevertheless, vesical diverticula of large size are occasionally found in women, and in exceptional cases have caused death. Pharyngeal diverticula (pharyngoceles). — Localized dila- tations of the pharynx are of three kinds : — Abnormal persistence and distension of certain pouches which, as a rule, exist in the embryo only — e.g. the pouch of Rathke and the branchial clefts. Pouching of the pharyngeal wall at its junction with the oesophagus. Protrusions (hernise) of the mucous membrane lining Rosenmiiller's fossa. When the pouch of Rathke persists it may dilate and form a cyst in the pharynx near the junction of its posterior wall with the roof. Such cysts have been known to attain the dimensions of a ripe cherry. In order to appreciate the nature of at least one form of pharyngeal pouch it will be necessary to take into consideration an interesting congenital defect to which the pharynx is liable. PHARYNGOGELES 613 It occasionally happens that children are born with what is known as an imperforate pharynx, that is, instead of the pharynx and cBsophagus forming a continuous tube, the pharynx terminates as a cul-de-sac near the level of the cri- coid cartilage. In such cases the upper end of the oesophagus terminates by opening into the trachea through its posterior wall. The Aperture by which the oesophagus communicates with the trachea. Fig. 324. — Imperforate pharynx. Fig. 325. — Pharyngeal diverticulum. {After Worthingtoii.) situation of the oesophago-tracheal fistula varies in different specimens ; sometimes it is as high as the third tracheal semi- ring, or it may be as low as the bifurcation of the trachea, and in at least one case it opened into the left bronchus. In most examples of imperforate pharynx the oesophagus is connected with the lower end of the pharynx by a fibrous band, which indicates that the two structures were originally continuous but that their continuity has been disturbed by secondary changes (Fig. 824). The constant association of an oesophago-tracheal fistula with imperforate pharynx indicates some relation between the 614 PSEUD0-GYST8 two conditions. The explanation which at once suggests itself is, that it may be due to some influence exercised by the pulmonary diverticulum which leaves that portion of the embryonic fore-gut ultimately represented by the oesophagus. This subject has been handled with remarkable acumen by Shattock. It is necessary to describe congenital imperfections at the junction of the pharynx and oesophagus, because it is at this point that pouches are apt to form. A typical example of a pharyngeal pouch, or pharyngocele, is shown in Fig. 325. The case is very carefully described by Worthington. The parts were obtained from a man 69 years of age. There was a stricture of the oesophagus at the level of the cricoid cartilage that would admit merely a urethral bougie. This obstruction ultimately led to the death of the patient. He could swallow food and retain it for a time ; it would then regurgitate. At the post-mortem dissection the pouch was detected ; it was in shape like the finger of a glove, and had a depth of 9 cm. and a circumference of 6 cm. The mucous membrane at the seat of the stricture was quite healthy. About two-thirds of the pouch was covered with muscle derived from the inferior constrictor. An examination of pharyngeal pouches such as exist in museums would lead the observer to believe that the orifice of communication between the pharynx and the pouch was circular ; but there is good reason to believe that it assumes a slit-like form even when the pouch is full of food. So far as our knowledge at present extends in regard to this variety of pharyngocele, it would appear that such pouches arise in all probability as congenital defects ; but it is important to remember that they rarely cause inconvenience until late in life. Thus, Ludlow's patient was 60, Worth- ington's 69, Chavasse's 49, and Butlin's 47. It is necessary to point out that a pharyngocele of the character represented in Fig. 325 arises in a different manner from that depicted in Fig. 246 ; the latter is probably due to a persistent bronchial cleft. Treatment. — Pharyngoceles are likely to be much more carefully studied in the future than they have been in the past, for the condition has on more than one occasion been TRACHEAL DIVERTICULA 615 correctly diagnosed, and the pouch removed through an incision in the neck and its slit-like orifice of communica- tion with the pharynx occluded by sutures — a manoeuvre that has been followed by complete success in the hands of Bergmann, Butlin, and others. (Esophageal diverticula. — Hernial protrusions of the mucous membrane of the oesophagus through the muscular coat are not common. They vary greatly in size. Some are no larger than cherries, others may attain the size of a closed fist. Diverticula arise in many parts of the oesojDhagus ; nothing is known as to their cause. Tracheal diverticula. — These are small hernial protrusions of the mucous membrane of the trachea ; they are uncommon, and invariably occur near the junction of the trachealis muscle with the cornua of the semi-rings of the trachea. Rokitansky regarded them as dependent on chronic catarrh of the trachea. Gruber, on the other hand, was of opinion that they are re- tention-cysts of the glands in the tracheal mucous membrane ; they are of little clinical interest. The tracheal divertimdum of the eimi. — The emu (DromcBus novce- hollandice) is normally provided with a tracheal diverticulum of great interest. In this bird there is a natural defect in the front of the trachea, at a spot varying between the fiftieth and sixty-fifth ring. The deficiency may involve six or more rings. In the emu chick the defect is scarcely noticeable, and the extremities of the rings are almost in contact. As the bird grows the tracheal mucous membrane becomes slowly herniated through the opening until it forms a huge sac between the skin of the neck and the trachea. The cyst- wall is composed of connective tissue with scattered bundles of striated muscle-fibre ; its mucous lining is directly continuous with that of the windpipe, and is dotted with the orifices of glands (Fig. 326). The adult emu inflates this sac when it produces the peculiar boom- ing sound which resembles the noise made by blowing across the mouth of a large bottle. This large tracheal sac may inflame and become distended with mucus. In a specimen which I secured and forwai-ded for preservation to the museum of the Royal College of Surgeons, London, the sac contained two pints of mucus. The bird was unfortunately droAvned in this fluid, for while I was making an attempt to evacuate the contents of the sac the fluid entered the opening in the trachea and suffocated it. Murie has written an excellent account of the anatomy of the trachea of the emu. I can confirm his observations, having enjoyed oppor- tunities of dissecting the adult emu and the emu chick. Concerning the function of this pouch nothing is known. 616 PSEUI)0-GY8T8 The guttural pouches of the horse. — In man the pharyngeal orifice of each Eustachian tube opens in relation with a bay or recess termed the fossa of Rosenmiiller. In the horse the tubes terminate in a very different manner. When the head is removed at the occipito- atlantal articulation, and the pharynx, with the associated structures, carefully dissected from the muscles on the ventral aspect of the cer- vical region of the spine, it will be found, as a rule, difficult to avoid Cul-de-sac. Wall of pouch Fig. 326. — Tracheal oi^euing and pouch of an emu. The pouch is cut so as to expose its interior. The surrounding feathers are cut short. {After Murie.) cutting into two large sacs separated from the atlas and axis by loose connective tissue. They reach to the base of the skull, extend downwards to the larynx, and send processes to occujiy the intervals between the long styloid processes and the mandible. These sacs are the guttural pouches ; they abut upon, but have no communication with, each other, and occupy the whole of the naso-pharynx. Each pouch is lined with delicate mucous membrane containing glands and furnished with ciliated epithelium. TEAGBEAL DIVERTICULA 617 The mucous membrane of the guttural pouches is directly con- tinuous with that lining the Eustachian tubes. The pouches them- selves appear as large saccular dilatations of the terminal ends of the tubes, and for this reason they are termed by some writers the Eustachian pouches. Each pouch opens into the pharynx immediately above the soft palate by a valvular orifice ; one side of the valve is formed by the leaf-like termination of the Eustachian tube. Of the functions of these pouches nothing is known. They are often a source of inconvenience to horses, for the mucous membrane is very prone to become inflamed, and the scanty outlet for the secretion leads to its Fig. 327. — Concretions from the guttural pouches of horses. {Nat. size. ) retention and the consequent dilatation of the sacs. When enlarged in this way they may have a capacity of six or more ounces each. The retained secretion may decompose, and the sac become distended with pus, which is discharged at intervals through the nose ; or the pharyn- geal orifice may be occluded, and the pouches enlarge to such an extent as to require an incision through the skin of the neck or through the mouth. Not infrequently the contents of the pouches become inspissated and formed into concretions. These are of different shapes and sizes, and vary in number from one, two, or three to fifty or even more. Generally they are of an oval shape ; not seldom they resemble beans. In consistence these concretions are like cheese, and on section have a laiuinated appearance. They are composed of mucus and inflammatory products mixed up with organic particles (Fig. ,327). The grit in these concretions enables an explanation to be offered concerning the liability of the pouches to attacks of inflammation. As the orifices of the pouches are in direct communication with the nasal 618 PSEUD0-CT8T8 passages, dust can easily enter them when snuffed up with fragments of hay, straw, dried seeds, and other organic and inorganic particles from dusty nose-bags and mangers. Laryngoceles. — In certain adult monkeys, particularly the chimpanzee {SimAa troglodytes), the deep cervical fascia is undermined by diverticula from the laryngeal mucous membrane. This large subfascial air-chamber communi- cates with the larynx through the thyro-hyoid membrane ; it extends downwards to within 2 cm. of the presternum. Exceptionally it dips into the anterior mediastinum, and laterally into the armpits, the axillary fasciae forming the lowest limits of the sac. In one fine chimpanzee I injected this huge reservoir, and found it would hold three pints of injection mass. In the howling monkey, Mycetes, the air-sac is very large, and the basi-hyal is hollovred to form a resonance chamber. Cervical air-sacs exist in many mammals, and can be inflated at will. They arise as diverticula from the larynx, either from the ventricle or from the pouch of Morgagni in the middle line of the larynx below the epiglottis. In the early stages the lateral pouch resembles the human sacculus laryngis inflated. Gradually the sacs undermine the deep cervical fascia and subsequently coalesce. The air-sac of the adult chimpanzee is formed by fusion of two lateral pouches and a niedian pouch. There is great variety in the degree of development of the cervical sacs in different genera and species of mammals. In 1888 I stated the following reasons for regarding some kinds of congenital cervical cysts in children as examples of laryngeal saccules : — The congenital nature of the cysts. Repetitions of animal structures of this kind are always congenital. Their relation to the hyoid bone and larynx. The hollow of the basi-hyal in man represents the large cavity in the basi-hyal of many mammals. The situations of the cysts beneath the deep cervical fascia and their occasional extension into the axillse. von Bergmann, E., " Ueber den OSsophagusdivertikel und seine Behandlung." —Arch.f. klin. Chir., 1892, xliii. 1. Butlin, H. T., "On the Removal of a ' Pressure-Fonch ' of the (Esophagus." — Med.- Chir. Trans., 1893, Ixxvi. 269. REFEBENGE8 619 Chavasse, T. F., " On a Case of Pressure Diverticulum Ox the CEsophagus." Trans. Path. Soc, 1891, xlii. 82. von Kostanecki, " Zur Kenntniss der Pliarynxdivertikel des Menschen mit besonderer Berucksichtigung der Divertikelbildungen und Nasenrachen- raum." — Virchow's Arclt.f.patli. Anat., 1889, cxvii. 108. Lane, W. A., " The Pathology of Extravasation of Urine and of Sacculation of the Urethra and Bladder." — Giu/s Hasp. Mepis., 1885-86, xliii. 29. Ludlow, "A Case of Obstructed Deglutition from a Preternatural Dilatation of the Bag formed in the Pharynx." — Medical Observation.'^ and Inquiries, 1769, iii. 85, pi. v. Shattock, S. G., " Congenital Atresia of the ffisophagus."— 2Va?is. Path. Soc. 1890, xli. 87. White, W. Hale, "A Sacculated Bladder in a Female."' — Trans. Path. Soc, 1883, xxxiv. 146. Worthington, W. C, "A Case in which a large Pouch was formed in the (Esophagus in connection with Contraction of the Canal." — Med.-Chir. Trans., 1847, xxx. 199. CHAPTER LX PSEUDO-CYSTS (Continued) STNOyiA.L CYST, GANGLION, AND BUaSA Synovial cysts. — Cysts containing synovia arise in three ways : — 1. Hernial protrusions ot the synovial membranes of j oints. 2. Burste in the immediate neighbourhood of joints. 3. Hernial protrusions of the s3^novial sheaths of tendons. Synovial cysts arise in connexion with the hip, knee, ankle, shoulder, elbow, and wrist joints. They have been most carefully studied in connexion with the knee-joint. The cysts form swellings, in some cases as large as an orange, situated near the knee-joint, usually in close relation with the tendons of the semimembranosus, biceps, or gastrocnemius muscle. Occasionally the cyst will be situated in the calf on the inner side, sometimes as much as 8 cm. below the knee. When the swelling is situated near the joint, pressure will cause it to disappear, the synovia it contains passing into the general cavity of the joint. When the cyst is situated at a distance from the joint, pressure upon it may have no effect in diminishing its size, because in many cases the communica- tion between the cyst and the joint-cavity is by a very narrow, almost capillary channel. The cysts arise usually in connexion with joints which are chronically diseased, and seem to be common in tuberculous joints. It is believed by those who have devoted special attention to these cysts that when the joints become distended with synovia the internal pressure causes the synovial mem- brane to protrude through weak spots in the capsule, the diverticula making their way along the intermuscular planes. 620 SYNOVIAL CYSTS 621 This mode of origin is similar to that which obtains in the case of sacculated bladders. It is also certain, for it has been demonstrated by dis- Opening of bursa into tlie joint. Bursa, Remains of a previous cyst. Fig. 328. — Bursa under the semimembranosus tendon commuaicating with the knee-joint. A cyst had been incised and drained sixteen months previously; its partially obHterated channel persists. {B'Jrcy Power.) section, that some synovial cysts are due to bursas normally existing under the adjacent tendons becoming abnormally large and communicating with the joint-cavity in consequence of absorption of the contiguous parts of the wall by pressure (Fig. 328), This seems to happen most frequently in the case 622 PSEUD0-GY8TS of the bursa under the semimembranosus. It does not necessarily follow, because an individual has a synovial cyst near the knee, that the joint is diseased ; attendance in an out-patient room will show that many synovial cysts slowly disappear without treatment. This is important to bear in mind, for interference with these cysts is, as a rule, needless and often productive of much harm. Aspiration, injection of iodine, and the insertion of setons may lead to suppuration, and destruction of the joint with which the cyst is connected. Morrant Baker, who first drew special attention to these synovial diverticula, states that when they arise in connexion with the knee the cyst will project in the popliteal space, the upper part of the calf, or on the inner side of the calf as much as 10 cm. below the head of the tibia. In the case of the shoulder, the cyst projects in front of the joint a little below the clavicle, or in the upper third of the arm in the course of the long tendon of the biceps. In the case of the elbow, the cyst projects on the inner side of the arm above the condyle. I have seen a cyst of this kind as high as the insertion of the coraco-brachialis, connected with the elbow-joint by a tubular process of the diameter of the anterior interosseous arterj^. When they arise from the carpal joints, the cysts project on the back or front of the wrist. (See under Ganglion.) When connected with the hip-joint, the cyst forms a swelling in Scarpa's space ; and in the case of the ankle, the bulging is most marked in front and to the outer side of the joint. The fluid contained in synovial cj/sts is in most cases identical with synovia, and occasionally contains " melon- seed" bodies. When the joint is tuberculous the fluid in the cyst will be purulent ; when the skin over these swell- ings is red and glossy they have been mistaken for simple abscesses and incised. Ganglion. — A ganglion is a cyst formed by the hernial protrusion of the synovial lining of a tendon-sheath. There are two species — simple and compound. A simple ganglion is seen in its most typical condition on the back of the carpus, where it forms a rounded sessile elastic swelling which becomes tense when the wrist is flexed, and partially, or wholly, disappears when the wrist is ex- GANGLIA 623 tended. Many of these swellings, which, are entered in clinical records as ganglions (or ganglia), are not connected with tendon-sheaths. I have satisfied myself by careful dis- sections that many of them are diverticula from the carpal joints, and in some instances they arise from the inferior radio-ulnar joint. As in the case of the larger joints, synovial cysts arising from the carpus are occasionally associated with tuberculous arthritis. Ganglia are sometimes met with on the fingers in con- nexion with the sheaths of the long flexors and on the dorsum of the foot ; as well as on the outer side of the ankle in relation with the tendons of the peroneus longus and brevis. The fluid in a simple ganglion is clear, transparent, and viscid, resembling apple jelly. The compound ganglion is a much more serious con- dition. It occurs mainly at the wrist in connexion with the flexor and extensor tendons ; it also occurs occasionally on the tendons of the peroneal muscles, where they lie in relation with the calcaneum. A compound ganglion at the wrist assumes an irregular shape and extends for a variable distance up the forearm ; it also sends a prolongation under the annular ligament to appear in the palm, when it arises in connexion with the flexor tendons ; a similar extension under the posterior annu- lar ligaments is usually noticed when a ganglion is connected with the extensor tendons. A compound ganglion is usually soft and elastic, and imparts a crepitant sensation to the examining fingers when the tendons are set in action. This crepitant sensation is due to the presence in the ganglion of small bodies familiarly known as melon-seed bodies, from their shape and consistence ; these are sometimes present in enormous numbers. There is much difference of opinion as to the source of these bodies ; often, in the course of an operation, they may be seen hanging from the inner wall of the ganglion. An examination of many of the loose bodies will show that they have slender stalks ; these appear more clearly when they are placed in water. Bodies identical in structure are met with in synovial diverticula and even in bursal sacs, particularly the prepatellar bursa. Treatment. — A simple ganglion, such as is so common on 624 P8EUD0-GYSTS the back of the wrist, is in a general way successfully treated by bursting it subcutaneously b}'' the direct pressure of the thumb, and then applying a graduated compress for a few days. When the wall is so thick that it will not rupture, the swelling may be punctured with a very narrow scalpel ; this allows the mucoid contents to escape, and the application of a firm compress for a few days will obliterate the sac. A compound ganglion should be dissected out as if it were a tumour, and it would appear that the patient runs less risk from this mode of treatment than by the common practice of incision and drainage. It is well to bear in mind that some of these ganglia are associated with the early stages of tuberculous disease of the wrist-joint, and a few are undoubtedly due to tuberculous infection of the tendon-sheaths. Bursse. — On many parts of our bodies where muscles and tendons glide over osseous surfaces, or in situations where skin lies in close contact with bony prominences, membran- ous sacs occur filled with glairy fluid; such sacs are known as bursse. Structurally a bursa consists of a thin-walled sac filled with glair}^ fluid. The inner wall of the cyst is quite smooth and, as a rule, devoid of epithelium. In certain situations, such as the anterior surface of the patella and the posterior surface of the olecranon, a bursa is normally present. Bursal sacs may form in any part of the subcutaneous tissues when the overlying skin is submitted to friction and intermittent pressure, as in talipes when the patient walks on the dorsum or side of the foot; beneath corns; and at the metatarso-phalangeal joint in the condition termed bunion. Such are called adventitious burssB. When burste arise in connexion with tendons, they are spoken of as subtendinous -bursse, and they often communicate Avith the sheath of the tendon, and even with an adjacent joint. The large bursa so constantly present at the insertion of the semi- membranosus often has a direct communication with the joint. The origin of bursal sacs has been explained in the follow- ing manner : — When the skin moves over joints or hard prominences the intermediate connective tissue becomes torn or ruptured, thereby leading to the formation of spaces in which fluid BURSAL 6'25 collects. The boundary walls are at first irregular, and are formed by adjacent connective tissue. Finally this becomes smooth and forms the sac-wall. Bursse may arise during intra-uterine life when the foetus is submitted to abnormal pressure. Many remarkable in- stances of this have been recorded, especially in association with talipes. Most subcutaneous and many subtendinous bursEe arise after birth. Bursae often attain an abnormal size in con- sequence of pressure associated with particular occupations. For instance, too much kneeling on hard material, whether by housemaids, devout persons, or carpet-layers, produces the familiar prepatellar bursa ; repeated blows on the elbow pro- duce miner's elbow ; from carrying weights on the shoulder porters are liable to get a bursa over the acromial end of the clavicle ; tailors from their cross-legged habit of sitting are sometimes troubled with one over the external malleolus ; whilst weavers and lightermen from prolonged sitting on hard seats suffer from bursas over their ischial tuberosities : soldiers when sleeping too frequently on the hard floor of the guard-room get them over their greater trochanters ; the pressure of ill-fitting boots develops a bursa over the enlarged head of the metatarsal bone of the hallux — when associated with partial dislocation of the first phalanx it is known as a bunion ; and bursse are quite common on the ends of ampu- tation-stumps, and in relation with exostoses. Clement Lucas has described as the needlewoman's bursa a cyst that formed on the palmar surface of the terminal phalanx of the middle finger in an old seamstress. A bursa is often present between the body of the hyoid bone and the thyro-hyoid membrane; and jockeys acquire one in front of the ankle from the pressure of the stirrup. Bursas are liable to intiame, a process that may lead to suppuration, or stop short of that condition and become chronic or recurrent and lead to secondary changes in the walls of the sac so that its cavity becomes almost obliter- ated. Chronically inflamed bursse may attain the size of fists, especially the prepatellar and ischial varieties. Pre- patellar bursas sometimes rapidly solidify in syphilitics. Jephson, in his interesting account of " Emin Pasha and 2 o 626 PSEUDO-CYSTS the Rebellion at the Equator," relates that the woraen and many men of the Bari tribe whom he saw working in the fields had enlarged prepatellar burste due to kneeling whilst at work, and to the fact that the entrances to their huts were so low that it was necessary to enter on the hands and knees. r-r/^o/^hjh Fig. 329. — Gravid Fallopian tube with a pseudo-cyst or capsule formed around the blood effused through the ccelomic ostium, b shows the capsule entire, and in a it is in section so as to display its relation to the ostium and fimbriae,*. Treatment. — An inflamed bursa demands rest and the local treatment usually employed for inflamed parts. When the bursa is distended with fluid, it is the custom to apply a plaster of mercury and ammoniacum over the swelling and fix it firmly with a bandage. It is probable that the firm compression is the chief agent in promoting the absorption of \BVB8^ 627 the fluid. In some cases the swelling subsides spontaneously, and this probably explains the supposed efficacy of the appli- cation of tincture of iodine. When burs£e are repeatedly irritated, the walls become so thick that the tumour has to be excised. This mode of treat- ment is necessary when a bursa contains loose bodies. When the bursa is situated over the patella, malleolus, ischial tuberosity, or trochanter its removal is a very simple pro- ceeding. When a bunion inflames and suppurates it may involve the underlying metatarso-phalangeal joint. Many of these cases, especially in elderly individuals, demand amputation of the toe. When it is necessary to carry out this measure, it is much more satisfactory to remove the metatarsal bone as well as the toe. Pseudo-cysts in connexion with the Fallopian tube. — When from any cause a clean foreign body finds its way into the peritoneal cavity, or a sterile coagulable fluid is exuded therein, a process is established whereby the foreign substance is encysted. Shattock once found a rounded body with a diameter of 6"25 cm. in the pelvis of a man, between the rectum and bladder. On section a piece of iron was detected in its centre, surrounded by regular laminse of structureless material. He regarded this as an instance in which a piece of metal taken into the alimentary canal had entered the peritoneal cavity by traversing the wail of the intestine ; it had then become encysted by exudation (lymph) from the peritoneum. It happens very frequently in cases of tubal pregnancy which terminate by what is known as tubal abortion, and especially the form known as incomplete tubal abortion in which blood slowly trickles, or even drips, from the coelomic ostium, that the effused blood becomes surrounded by a lowly organized capsule, and this is occasionally so complete as to appear like an ovoid bulb or amphora containing blood, and its neck embracing the coelomic ostium of the tube (Fig. 329). This condition, and the mode of formation of the cap- sules, have been particularly studied by Saenger, Taylor, and Handley. The last observer has proved that capsules 628 PSEUDO-CYSTS of this kind are also formed occasionally in connexion with tubal pregnancy terminating by rupture. Capsules of this nature occasionally form around sterile inflammatory effusions (Fig. 330). It would appear that the conditions necessary to the production of these capsules are these : the intruded product ■ — whether a solid body, an infusion of blood, or coagulable inflammatory fluid — should be free from pathogenic organ- isms, and, in the case of fluid, be slowly effused. In this way imperfect capsules are formed on the walls of ovarian cysts, especially dermoids, and there can be little Fie 330. — Fallopian tube and ovary ; the ccelomic ostium and fimbriee are enclosed in a capsule of new formation. From a case of acute salpingitis. doubt that many of the reported cases in which these cysts are stated to have burrowed between the layers of the broad ligaments rest on erroneous observation, and that the supposed investment by the mesometrium was in reality a capsule of new formation. The most perfect capsules formed in this Avay are met with around echinococcus cysts in the belly, especially those which project from the under-surface of the liver, or grow in the meshes of the omentum, and on occasions they may be very thick. This explains how echinococcus colonies in the belly are provided with thick spurious capsules, whereas those growing in the cerebrum have none. CHAPTER LXI PSEUDO - CYSTS (Continued) NEURAL CYSTS Under this heading it is proposed to consider a number of conditions, some of which, like hydrocephalus and one variety of spina bifida, should be described with tubulo-cysts. Other varieties of spina bifida should be discussed with diverticula. On the whole it is more convenient to consider them collec- tively as neural cysts. Hydrocephalus. — This term is applied to the head when abnormally enlarged in consequence of excessive accumula- tion of fluid in the ventricles of the brain. A very large majority of cases are congenital, or commence in the early months of infancy. Occasionally the condition will arise at a later period of life, when the fontanelles are obliterated ; expansion of the skull is then impossible. Hydrocephalus very frequently accompanies spina bifida. Yery many hydro- cephalic foetuses die during delivery, the large size of the head hindering its successful transit through the maternal passages. In some cases the head ruptures in consequence of the pres- sure to which it is subjected, or it is intentionally perforated. In many cases of hydrocephalus which survive delivery, the distension is only slight at birth. The frequency with which hydrocephalus and hydramnion co-exist w( uld indicate that the association is something more than mere coincidence. Statistics respecting the frequency of hydrocephalus drawn from living children are untrustworthy, as prenata) hydrocephalus is very fatal. In typical cases of hydrocephalus attention is arrested by the large size of the cranium and the smallness of the face. This is due to the slow accumulation of fluid within the cerebral ventricles distending them and causing wide separa- tion of the cranial bones, whilst the bones of the face retain 629 630 P8EUD0-CY8TS their natural proportions. The two halves of the frontal bone are separated from each other ; the spaces between the parietal bones, and between these and the occipital, are far wider than usual (Fig. 331). Indeed, the bones of the cranial vault are so separated from each other, whilst those of the base retain their usual juxtaposition, that the bones of a hydrocephalic skull were compared by Trousseau to the petals of an opening flower. 331. — Skull of an infant the subject of hydioceplialus. {Museum, Middlesex Hospital.) The head may become so large as to attain a circumference of a metre, or even a metre and a half when measured hori- zontally — that is, from the superciliary ridges to the inion The bones are excessively thin, and consist of a single table. The vault presents large membranous spaces irregularly dotted with ossific deposits. The sutures in relation with the parietal bones are occupied with Wormian bones ; as many as two hundred have been counted in one skull (Fig. 332). In hydrocephalics who attain adult life the skull may become completely covered in with bone. The brain presents great changes. The lateral ventricles are widely distended, and the crura cerebri, corpora striata, EYDBOGEPHAL US 631 optic tlialami, and other structures in the base of the brain are flattened. The cerebral hemispheres form thin boundaries to the ventricles, often less than 10 mm. in thickness ; the convokitions become obhterated. In nearly all the speci- mens the distension is limited to the lateral and third ven- tricles ; occasionally the fourth ventricle also is distended (Fig. 333). In some specimens each lateral ventricle has been Fig. 332.— Skull of an adult the subject of hydrocephalus. {Museum., Middlesex Hospital.) known to attain a length of 20 cm. and to communicate with its fellow through an opening three inches wide. When the ventricles are very distended and the skull is proportionally thin, a wave of fluctuation may be trans- mitted from side to side. In exceptional cases the head is translucent. In an account of hydrocephalus it is difficult to avoid reference to the classical case of James Cardinal, especially as a cast of his head is to be found in many pathological museums (Fig. 334). James Cardinal died at the age of 29 years in Guy's 632 P8EUD0-C'Y8T8 Hospital, under the care of Sir Astley Cooper, in 1824. He was born at Coggeshall, Essex, in 1795. At birth his head was very little larger than natural. A fortnight later it began to increase, and gradually grew until he was five years old, and then appeared to remain stationary. He was unable to walk until 6 years of age, but went to school and learned to read and write. His head was at this period translucent Fig. 333. — Sagittal section of a child's head the subject of hydrocephalus, with the brain in situ. The head of the arrow is in the fourth and its feathers in the third ventricle. The iufundibulum is widely dilated. (JlKseiim, Middlesex Hospital.) when placed between the eye of the observer and a bright light. Cardinal continued in tolerable health until 23 years of age, when he began to have fits, for which he applied to the hospital. His manners were childish, otherwise his mental faculties were well developed. Death eventually supervened from lung disease. When the head was examined the brain was found lying at the base of the skull. Between the membranes there were HYDROCEPHALUS 633 seven pints of fluid. The ventricles contained, one pint. It appeared as if the fluid had been originally contained within the ventricles, but had burst through an opening in the corpus callosum and compressed the brain downwards. The cranium measured 82'5 cm. (33 inches) in circumference, and had a capacity of ten pints. The skeleton is contained in Guy's Hospital Museum. The fluid in hydrocephalus is identical with cerebro-spinal Fig. 334. — Drawing from a cast of the head of James Cardinal. The cast from which this drawing was taken appears to have been moulded April 11th, 1822. fluid. Occasionally it has been found to contain albumin. This may be attributed to inflammation, and has been observed in those cases where paracentesis has been performed. The amount of fluid may be very large. Six and eight and even ten pints have been recorded. Little is known as to the cause of hydrocephalus. In many cases obstruction to the interventricular communications has been detected. Hydro- cephalus is often associated with spina bifida, and all the passages in the brain and the central canal of the cord have been found dilated. 634 PSEUDO-CYSTS The great difficulty encountered in investigating the pathology of this condition arises from the soft and diffluent nature of the brain of hydrocephalic foetuses, especially when stillborn. It should also be remembered that many gross malformations of the limbs and viscera are often associated with hydrocephalus, and it is well to bear in mind the fre- quency with which it is accompanied by hydramnion. Hydrocele of the fourth ventricle. — ^Leading from each lateral angle of the fourth cerebral ventricle there is a tubular process encircled by a duplicature of the ligula termed the cornucopia. These passages or lateral recesses are traversed by the choroid plexuses of the fourth ventricle, and the re- cesses themselves open into the subarachnoid space at the base of the flocculus, close beside the root-filaments of the facial, auditory, glosso-pharyngeal, and vagus nerves. These passages establish free communication between the fourth ventricle and the general subarachnoid space. When one of these processes becomes occluded, the recess will dilate and form what Virchow terms hydrocele of the fourth ventricle. This pathologist has figured a specimen that had attained the size of a cherry-stone ; it pressed upon the flocculus and the facial nerve ; remnants of the choroid plexus of the fourth ventricle projected into the cyst. Though the walls of this cyst were thin, its pressure had caused paralysis of the facial nerve. Cranial meningocele. — This term is applied to a hernial protrusion of the meninges of the brain through an unossified portion of the skull. When the protrusion consists of brain- matter as well as membranes it is described as a meningo- encephalocele. Meningoceles, using the term in its general sense, occur in definite regions. The commonest of all situations is the occiput ; in about two-thirds of the cases the tumour projects in this part of the skull. Next in frequency to their appear- ance at the occiput, meningoceles appear at the root of the nose. In other regions of the skull they are excessively rare. It is usually stated that they may appear at the anterior fontanelle, but critical examination of the descriptions of sus- pected cases makes it probable that many of the supposed meningoceles were dermoids (p. 460). CRANIAL MENINGOCELE 635 Occipital meningoceles appear, during life, to protrude through the foramen magnum ; when the parts are dissected the pedicle will be found to make its way through a gap in the supra-occipital between the posterior margin of the fora- men magnum and the occipital protuberance. This space during early embryonic life is occupied by a fontanelle. The relations of the flocculus in cases of occipital meningo- cele are of importance. In the descriptions of reported cases of this malformation the cerebellum, if referred to, is described as rudimentary or absent. As a matter of fact, in these cases the cerebellum is absent, and that which is supposed to represent this part of the brain is an enlarged flocculus (Fig. Flocculus. Fig. 335. — Occipital meningo-encephalocele. The cyst probably represents an ex- panded fourth ventricle : there was no cerebellum, but a large and conspicuous flocculus. 335). Cleland has pointed out that the flocculus is developed from a lateral outgrowth of the floor of the third encephalic vesicle, whilst the cerebellum is developed from the foremost part of the roof of that vesicle. An appreciation of this fact throws valuable light on the nature of occipital meningocele, for the absence of the cerebellum indicates that the hernial protrusion is the third encephalic vesicle ; instead of its walls thickening to form a cerebellum, they become pas- sively dilated into a cyst. Indeed, this form of meningocele bears much the same relation to the fourth ventricle and the cerebellum that hydrocephalus bears to the lateral ventricles and the cerebrum. An occipital meningocele might not inaptly be described as hydrocephalus limited to the fourth ventricle. 636 PSEUDO-CYSTS Occipital meningo-encephaloceles often hang so low as to render it difficult to decide whether the cyst belongs to the cranium or to the cervical region of the spine. There is reason to believe that the pedicle of a cranial meningocele may become obliterated so as to cut off the communication between the cyst and the subdural space. I have never had an opportunity of dissecting a specimen in which this has happened. Such an event certainly occurs with spinal meningoceles. A cranial meningocele is sometimes associated with spina bifida ; such a combination is, as a rule, accompanied by gross malformations, especially in connexion with the lower limbs. It has already been mentioned that dermoids are apt to be mistaken for meningoceles, and it is certain that meningoceles are sometimes mistaken for dermoids. With careful aseptic precautions, meningoceles may be, and often are, safely excised. In some cases treated in this way hydrocephalus has supervened. Infants with meningoceles, especially when the cyst is large, rarely survive their birth many weeks ; death is usually due to sloughing of the sac and septic meningitis. The cerebro-spinal fluid. — Hydrocephalus and meningo- cele are due to an excessive accumulation of this fluid, which has physical and chemical properties that dis- tinguish it from any other secretion in the body. Under normal conditions it is clear like water, with a specific gravity of 1006 to 1008, and devoid of all corpuscular ele- ments ; it contains a small amount of protein matter. Its principal constituent is sodium chloride ; it also contains traces of carbonates, bicarbonates, phosphates, urea, and dextrose. The presence of sugar (dextrose) was detected by Claude Bernard (1858), and this observation is confirmed by Mott and Halliburton. The quantity of fluid contained in the ventricles and subarachnoid spaces of the brain and the corresponding spaces of the cord is about 100-130 c.c. Judging from the way it gushes out when the membranes are punctured by a needle and cannula in the lower lumbar region, it exists under considerable pressure. The sources of this fluid are the choroid plexuses of the ventricles. These villous structures are familiar to all anato- GEBEBR0-8FINAL FLUID 637 mists. They consist of tufts of blood-vessels surroimdecl by- loose connective tissue covered by a single layer of cubical or spheroidal epithelium resting on a basement membrane. These tufts are well supplied with fine nerves. Although nothing is known with certainty on the point, it is supposed that the cerebro-spinal fluid exercises mechanical functions ; as the whole of the central nervous system is contained in a closed membranous sac, all the space which is not occupied by tissue, or blood, is filled with, it. The intraventricular spaces communicate with the sub- arachnoid spaces at the lateral angles of the fourth ventricle ; the actual lopenings are known as the lateral recesses. In spite of much careful dissection I have failed to detect the opening at the lower part of the fourth ventricle known as the foramen of Magendie. Mechanical interference with the intraventricular communications will lead to distension of the lateral ventricles. CHAPTER LXIl PSEUDO-CYSTS (Concluded) NEURAL CYSTS (Concluded) Spina bifida. — The term spina bifida is applied to congenital defects in the union of the laminae of one or more vertebrae, Central canal of the cord. ,^^, - Expanded nerve .iS; tissue. Fig. 336. — Lumbar region of a fcstus with spina bifida, variety myelocele. {After Shattoch.) {Museum, Middlesex Hospital.) associated with malformation of the sj)inal cord or its membranes. The spinal cord and a large part of the bram are formed by the dorsal coalescence of the medullary folds. The fusion of these folds commences in the thoracic and extends into the cephalic and caudal regions. For a short time after coalescence the embryonic cord and superficial epiblast re- main in contact. Gradually they become separated by the intrusion of connective tissue, some of which chondrifies and 638 MYELOCELE 639 afterwards ossifies to form vertebrae and intervertebral discs. In the early stages the cord has a longitudinal extent equal to that of the notochord, and this equality is maintained for some time after the closure of the medullary groove. Subse- quently the vertebral column grows at a greater rate than the nerve- tube ; the result is that at birth the medullary cone at the end of the cord is opposite the upper border of the second lumbar vertebra. The varieties of spina bifida are determined according to the stage of development at which the defect occurs, as de- termined by the anatomy of the parts. They are — 1, Myelocele ; 2, syringo-myelocele ; 3, meningo-myelocele ; 4, meningocele ; 5, masked spina bifida {spina bifida occulta) Fig. 337. — Diagram to represent the microscopic characters of a transverse section of a myelocele. 1. The 7nedv2lary folds mcty unite imperfectly and give rise to a myelocele (Fig. 336). In this case the cord is normally formed in the cervical and thoracic regions, but in the lumbar portion the central canal suddenly opens on to a shallow depression, the sides of which are slightly intumescent and then become gradually continuous with the skin. The tissue surrounding the furrow represents the medullary folds and consists mainly of very vascular nerve-tissue. When fresh this area is bright-red and resembles a nsevus. When this red tissue is carefully dissected from the underlying vertebrae and prepared for the microscope, it will exhibit on each side of the furrow nerve-cells embedded in neuroglia intermixed with plexuses of arterioles, venules, and 640 PSEUnO-GYSTS capillaries (Fig. 337). It is hard to determine the existence of epithelium on the surface of myeloceles, because there is usually some inflammation, and occasionally sloughing. Myeloceles are, according to my observations, more com- mon in the stillborn than in children who survive their birth a few days. Children with myeloceles rarely live more than a few days. This variety of spina bifida is often associated with defects in other regions of the body, including the brain which are incompatible with life. 2. The medullary folds unite througJiout, hut fail to separate from, the surface epiblast. The central canal hecomies subsequently dilated : syringo-myelocele. Syringo- myelocele is a rare variety of spina bifida, and cannot be distinguished from simpler forms during life. When the parts are dissected the nerves will be found passing round the convexity of the cyst (Fig. 338). Although syringo-myelocele is rare in a typical form, it may occur in combination with a men- ingocele. Glutton has carefully described an example (Fig. 339). 3. The cord is normcdly closed, but, before it separates from the surface epiblast, becomes compressed, by a collection of fluid within the meningeal spaces : mening-o-myelocele. " Probably two-thirds of all cases of spina bifida that survive birth are meningo-myeloceles. The condition is easily recognized ; there is a deficiency in the arches of the vertebrae, usually in the lumbar region, occupied by a cyst of variable size. Unless inflamed, or flaccid in consequence of leakage, the cyst is translucent and often presents a pink tinge. Its most posterior part is somewhat flattened, and occasionally a shallow median groove is seen. In some specimens, quite in the centre of the cyst there is a small umbilicus marking the central canal of the cord. At the edge of the cyst, where its walls become continuous with the skin, the margin is slightly raised, and immediately beyond Fig. 338.— Syringo-myelocele in transverse section. SPINAL MENINGOCELE 641 this tlie skin, even in tlie new-born, may present a circle of long hairs. Meningo-myeloceles are often associated with hydrocepha- lus and, in a large proportion of cases, with double talipes equino-varus and other severe deformities of the lower limbs. On transverse section of a meningo-myelocele the cord is found flattened on the posterior wall of the cyst like a strap, Avhilst the nerves reach their respective foramina by directly traversing the cavity of the cyst (Fig. 340). Sac of tlie meningocele. Fig. 339. — Syi'ingo- myelocele and meningocele in longitudinal section ; from the cervical region. [After Glutton.) That the strap-like band of nerve-tissue on the posterior wall of the sac is the flattened spinal cord was demonstrated by Shattock. .He cut sections of this part of the cyst and detected the central canal (Fig. 341). 4. The cord is normal, hut there is a local hernia of the membranes : meningocele. Protrusion of the membranes unaccompanied by the cord is by no means common in spina bifida. Although it has been met with in the cervical region of the spine, it most frequently affects the lumbo-sacral region, or may be con- fined to the sacral portion of the spine. Some writers on this malformation believe that the hernial protrusion may make its way between the arches of two vertebrse instead of 2 p 642 PSEUDO-CYSTS between the laminte of a single vertebra. It is a fact that the sac of a meningocele sometimes emerges through a very narrow orifice, and in a few instances this causes the cyst to become more or less pedunculated, and may lead to occlusion of the aperture by Avhich the dural space and the cyst com- municate and thus isolate the cyst. Virchow investigated a remarkable specimen ilhistrating this process. The patient was a negro child born with a large tumour pendulous from its buttock (Fig. 342). The tumour was removed in Central Africa and sent to Virchow, under the impression that it was a fatty tumour. Dis- section revealed a central space in the tumour lined with dura mater, which was covered with fat intermixed with muscle-tissue. The structure and arrangement of the parts were such as to lead Virchow to the opinion that the tumour was the sac of a meningocele (Fig. 343). A tumour in many respects similar to this, save that it I Fig. 340. — Diagram showing a meningo-myelocele in trans- verse section. The cord is flattened on the posterior wall of the cyst, and the nerves traverse its cavity. Fig. 341. — IVIicroscoirical appearances of the nerve-tissue from the wall of a meningo- myelocele showing the central canal. {Jifter Shaitock.) occurred in the cervical region of the spine, was removed by Solly in 1856 from a woman 27 years of age. The description of the case is accompanied by an exceedingly interesting clinical history, Pj^otrusions of dura mater uii- SPINAL MENINGOCELE US accompanied by cord or nerves (meningoceles) are more common in the sacral region than elsewhere. In some instances the membranes emerge through the deficiency (hiatus sacralis) normally present below the third sacral vertebra. This will perhaps be the most convenient place in which to refer to an abnormal disposition of the cord which I have Fig. 342. — African child with a pedunculated tumoui- (an occluded spiua bifida sac) attached to its buttock. (After Virchoiv.) met with in association with spina bifida. It is well known that in the early embryo the cord extends the whole length of the vertebral column, but at birth the apex of the medullary cone is on a level with the upper border of the second lumbar vertebra. I have placed in the museum of the Middlesex Hospital a spine with a large meningocele m the sacral region ; the cord runs the whole length of the neural canal and terminates near the tip of the sacrum (Fig. 344). 6U FSEUDO-GYSTS 5. The cord and its memhranes are normally formed, but the arches of one or more vertebrce are defective. There is, hoivever, no protrusion of the membranes or cord : masked spina bifida (spina bifida occulta). This defect, as it is unaccompanied by a cyst, is very apt to be overlooked. An interesting feature usually associated with the condition is an abnormal growth of hair in the loins. Hair-fields of this description may be localized to the loin, as in the original case described by Virchow, and the hair may form a long tuft (Fig. 345). In exceptional cases an abnormal Fig. 343. — Tumour from the African child (see Fig. 342), shown in section. growth of hair may extend from the loins over the buttocks and for a considerable distance down the thighs. The two varieties observed in the distribution of hair in these cases are well illustrated by the arrangement adopted by artists and sculptors in their representations of fauns and the goat-footed satyrs (Fig, 346). Many cases of spina bifida in addition to the " masked " species are accompanied by an excessive development of hair in the loin. Attention has also been drawn to the fact that a circlet of hairs is often observed on the skin immediately l)ordering the sac of a meningo-myelocele, even in new-born SFINA BIFIDA 645 babes. Hair-fields associated with occult spina bifida are sometimes mistaken for hairy moles, and surgeons have in some instances Avith misplaced industry removed them under that im]3ression. This excessive develoj^ment of hair (hypertrichosis) associated with defective closure of the neural arches is interesting when studied in connexion with the luxuriant growth of feathers on the heads of Polish fowls, for in many of these birds there is defective ossifi- cation of the bones of the cranial vault. In contrast, it should be mentioned that the bone beneath the exquisite crest of the crowned crane is abnormally thick. An important condition often associ- ated with spina bifida occulta is perforating ulcer of the foot. In- deed, this association is now so well recognized that in every case of perforating ulcer of the foot, occurring in young patients, it is the duty of the surgeon, as a matter of routine, to examine the loins. In addition to non-union of the arches in the vicinity of spina bifida, the vertebrae are liable to be defective in other ways, and of these defects the most striking is the absence of half a vertebra — that is, half the centrum, with its pedicle, lamina, transverse, articular and spinous processes, is wholly wanting. The persistent half of such a vertebra has the characters shown in Fig. 347, and is often ankylosed to the vertebra above and below. Sometimes the half-vertebra is in excess of the ordinary number. Exceptionally, a considerable Fig. 344. — Spinal column in section with a sacral meningo- cele. The spinal cord is re- tained in the sacral section of the neural canal, its embryonic position. From a child aged 3 months. 646 PSEUDO- CYSTS extent of the column will be replaced by an alternating series of lialf-vertebree ; this is especially seen when the cer- vical portion of the column is the seat of spina bifida. Half-vertebra3 occur occasionally independently of spina bifida ; they have also been detected in the spines of snakes, calves, fish (sole), and rabbits. The aniount of disturbance sometimes caused in a vertebral column b}^ spina bifida is very remarkable. Occasionally, horizontal ]3rocesses of bone project from the vertebral centra into the neui'al canal, and some- times transfix the cord. Several examples have been carefidly described in which the cord has bifurcated and coalesced again in order to enclose a beam of bone crossino' the canal in a sagittal direction. Complications of spina bifida. — Unfortunately all species of spina bifida are apt to be associated with other se- rious conditions, such as talipes ecjuino-varus, single and double and other gross deformities of the legs, hydrocephalus, men- ino'ocele, and malformations of the alimentary canal, such as imperforate aims and, on rare occasions, imperforate pharynx. Very exceptionally these two imperforate conditions of the ali- mentary canal have co-existed. The most serious complication of spina bifida is hydro- cephalus (p. 629). The ventricular cavities of the brain may be abnormally dilated at birth : in many cases the hydrocephalus slowly develops during the first few weeks of infant life, and the head gradually assumes enormous dimensions. In a small proportion of cases the sac of the spina bifida spon- taneously shrinks; coincidently with this the fontanelles gradually widen and hydrocephalus develops. I have in Fig. 345. — Hair-field overlying a spina bifida occulta ; there is also a long tuft in the cervical region. (FiscJier.) SPINA BIFIDA 647 several children seen liydroceplialus supervene when the sac in the loin has been made to shrink by artificial means. We have now to consider the various modes by which spina bifida destroys life. Of all the varieties of this malfor- mation, myelocele is the most fatal. A very large proportion of fcetuses in which this condition is present are stillborn; the few that survive their birth rarely live longer than three da3^s, the continual leakage of cerebro-spinal fluid being sufficient to explain the invariable brevity of their lives. Fig. 346. — ^gipan sporting with a faun. (Bacchus and Sileuus.) When a distinct sac is present, life may be prolonged many weeks, even when the sac-wall is thin ; Avhen it is thick life may be prolonged several years ; and when it is com- pletely skin-covered, some of these children survive and grow up to be healthy men and women. The prospects of each particular case are largely influenced by the thickness of the sac-wall and the absence of complications, especially hydrocephalus. In many cases, especially when the walls of the cyst are thin, the tissue is apt to slough — an event that allows the 648 PSEUDO-CYSTS sudden escape of the cerebro-spinal fluid and may terminate the hfe of the child in a few hours. Children often survive this accident, to succumb seven or ten days later to septic meningitis. Exceptional!}'', I have observed children recover from rupture of the sac and, escaping meningitis, slowly die from hydrocephalus. Occasionally the sac in the loin and the hydrocephalus will increase simultaneously. In such a case pressure on the anterior fontanelle will increase the tension in the spina bifida sac, and vice versa. Fig. 347.— Half -vertebra. {After Shattock.) The duration of a child's life with spina bifida, excepting the " masked " species, is very uncertain ; it is often prolonged when the nurse and mother are careful and vigilantly pre- serve the sac from injury. That spina bifida is a serious affection may be gathered from the figures in the Registrar-General's Reports ; about 800 individuals in England die from it every year. This in- formation is not precise, as the actual number of cases is much greater, the birth of the stillborn not being regis- tered. iSiO facts are accessible that will enable an estimate to be formed of the real frequency of the malformation. Treatment. — This has undergone a great change in re- SPINA BIFIDA 649 cent years. Instead of the slow and uncertain method of injection with iodo-glycerine solution, it has been shown that spina-sacs may be safely excised. The evolution of the central nervous system. — The extraordinary frequency with which the membranous and bony coverings of the central nervous system are malformed Fig. 348. — Meningeal lipoma simulating a spina bifida in a child 8 months old. {After Temoin.) induced me some years ago to investigate the abnormalities collectively classed under the term spina bifida, with the hope of obtaining some light as to the mode of evolution of the brain and spinal cord, for, as I pointed out in 1886, the pathological behaviour of the central canal of the cord indi- cated that it was an obsolete passage. In 1887 I came to the conclusion from embryological and pathological data that the brain and cord were in all probability evolved from a 650 PSEUDO-CYSTS segment of the lyrimitive intestine. This view has been con- firmed since by the inde^Dendent researches of Gaskell. Andriezen demonstrated the existence in amphioxus and ammocoetes of an epithehum-Uned duct extending from the buccal cavity into the ventricle (thalamocele), and suc- ceeded in transmitting carmine particles suspended in water into the central canal of the cord. His view is that the central canal of the cord is a remnant of the water- vascular system. Tails. — This account of spina bifida would be imperfect without a brief notice of tails, real and supposed, in the human subject. We may with Virchow arrange tails in two classes, true and false. True tails may be complete or incomplete : the most perfect or complete tails contain bony segments (vertebrse), as in the case of cats and dogs ; the less perfect or incomplete tails are like those of pigs^ soft and flexible. No one has yet reported an example of a tail in the human subject containing bony elements. Several cases have been investigated in which an appendage 5 cm. long, and soft like a pig's tail, has been found directly continuous with the coccygeal vertebrte. Most of the cases reported as tails were nothing more than examples of congenital sacro-coccygeal tumours, or a tuft of hair covering a masked spina bifida. Tumours sup- posed to be tails were in some cases dermoids ; in others fatty tumours (Fig. 348), or the sac of a spina bifida, and in many cases teratomas. Andriezen, W. L., "The Morphology, Origin and Evolution of Fanction of the Pituitary Body, and its relation to the Central Nervous System." — Brit. Med. Journ., 189i, i. 54. Bland-Sutton, J., "On the relation of the Central Nervous System to the Alimentary Canal: A Study in Evolution." — Brain, 1888, x. 429. Glutton, H. H., " Large Cervical Spina Bifida undergoing spontaneous cure." — Trans. Clin. Soc, 188G, xix. 99. Evans, Z. H., Intermit. Journ. of Surrj., 1895. Gaskell, W. H., " On the relation between the structure, fanction, distribution, and origin of the Cranial Nerves ; together with a theory of the origin of the Nervous System of the Vertebrata." — Journ. of Phys., 1889, x. 153. Humphry, Sir George, " Six specimens of Spina Bifida, with bony projections from the bodies of the vertebrae into the vertebral canal." — Journ. of Anat. and Phys., 1886, xx. 585. BEFEBENGES 651 NicoU, J. H., " The Operative Treatment of Spina Bifida and Hydrocephalus." — Glasgow llos]}. Re])ts., 1899, ii. 297. Shattock, S. G., " Eeport of a Committee of the Clinical Society of London nominated to investigate Spina Bifida and its treatment by the injection of Dr. Morton's lodo-glycerlne Solution." — Trans. Clin. Soc, 1885, xviii. 355. Solly, Samuel, '• Case in which a large Cyst was successfully removed from the upper part of the neck of a young woman aged 27, who was con- sidered at birth to have been the subject of Spina Bifida." — Mecl.-Chir. Trans., 1857, xl. 19. Virchow, 'Rudolf.— Zeitsohr./. Ethnologie, 1875, vii., 280, t. xvii. Fig. 2. Virchow, Rudolf, " Schwanzbildung beim Menschen." — Serl, Min. Woch., 1884, xxi. 745. Virchow, Rudolf, " Ueber einen Fall von Hygroma cysticum giutjeale con- genitum." — Virchow's Arclt.f.jrath. Anat., 1885, c. 571. CHAPTER LXIII ECHINOCOCCUS DISEASE (HYDATID CYSTS) The term hydatid formerly covered a large number of pathological productions, but is now restricted in human path- ology to the cystic stage of the tapeworm Tcenia echinococcus. This cestode, which in its mature form inhabits the intestines of dogs, is about 4 mm. in length and consists of four seg- ments, of which the fourth is larger than the rest of the body and is the only segment that becomes mature (Fig. 349). In referring to T. echinococcus in the mucous membrane of the dog's intestine, Leuckart writes that it " occurs in considerable numbers, some- times in many thousands, between the villi, so that only the milk-white proglottides project." It has been calculated that a ripe proglottis of this cestode contains about 5,000 ova. This is a good example of the prodigality of nature in all that concerns eggs, both animal and vegetable. The eggs of this worm are passively conveyed with either food or water into the alimentary canal of man, where they are hatched ; the embryos migrate from the intestine into some vascular organ or tissue, or, by gaining entrance into a blood-vessel, are passively conveyed into some distant part of the body and become transformed into cysts. The degree of infection depends upon the number of ova swallowed, and the ex- traordinary multitude of cysts and colonies found in some patients would suggest that an entire proglottis had been ingested. The cyst-wall has a peculiar structure ; it consists of an external highly elastic, lamellar cuticle, and an internal lining 652 X,(2 Fig. 349.— Tsenia echinococcus. {Leuckart.) BROOD-CAPSULES 653 consisting of sfranular matter, cells, muscle-tissue, and a water- vascular system (Fig. 350). The inner lining is often referred to as the parenchy- matous layer. In addition to the proper tissues of the cyst, there is often a more or less complete fibrous capsule, especially when the cyst projects into the peritoneal cavity. The mode by which these adventitious capsules are formed is discussed on p. 628. The true cyst is maintained in apposition with the fibrous capsule by the pressure of the contained fluid ; when this is removed by the abstraction or escape of the fluid, the mother-cyst at once collapses. The fluid is clear, limpid, colourless or slightly opalescent ; specific gravity, 1004 to 1015; it contains chloride of sodium, Fig. 350.— Small echinococcus cyst, showing the peculiar lamination of its wall. {Lenckaft.) succinic acid, and occasionally, in cysts situated in the liver, leucin, tyrosin, and sugar. Hooklets and scolices are also found. When the hydatid attains the size of a walnut, small vesicles or brood-capsules develop from the parenchymatous layer. These brood-capsules develop numbers of heads or scolices. The scolex when fully developed is about 3 mm. long, is furnished Avith four sucking discs and a rostellum of tiny blunt hooklets ; it has a water-vascular system and numerous calcareous particles. The fore part of the scolex can be withdrawn into the hinder part ; indeed, this is the position in which they are usually found (Fig. 351). As fresh brood-capsules and scolices are formed, the cyst enlarges, and, when seated in an organ or cavity of the body which imposes little restraint upon its growth, it may attain 654 HYDATID GTSTS enormous proportions — e.g. hydatid cysts of the liver have been known to acquire a capacity of sixteen pints. In many hydatids daughter-cysts are formed from brood- capsules and probably from scolices. Cysts containing large numbers of these translucent thin-walled vesicles are known as echinococcus colonies. Occasionally cysts even of large size do not contain vesicles or brood -capsules ; such are said to be sterile. The walls of sterile hydatids exhibit the characteristic lamination, and this enables the nature of the cyst to be recognized in otherwise doubtful cases. Echinococcus multilocularis (Virchow). — This is an ex- ceptional mode in which echinococcus disease manifests itself, Fig. 351. — Portion of the cyst-wall of an echinococcus colony, showing scolices. {Leiickart.) or, as Ziegler thinks, a distinct species. In this condition the vesicles are of small size, but occur in great number, and are not contained in a mother-cyst. The vesicles in such cases rarely exceed a pea in size, but the majority are much smaller ; very many are no larger than millet- or rape-seed. This variety occurs most frequently in the shafts of long bones; it has also been observed in the spinal canal. The multilocular hydatid occurs in the liver as a firm tumour; on section it presents trabeculse of dense fibrous tissue causing it to assume an alveolar appearance. The alveoli contain a gelatinous substance in which the shrunken vesicles are embedded. Most of these minute vesicles are sterile, but here and there a few booklets can with patience be demonstrated, Virchow was the first to detect the hydatid nature of such HYDATID BASH 655 tumours in the liver ; previously they had been described as colloid cancer. In very rare instances contracted and shrunken vesicles embedded in gelatinous material and sur- rounded by a distinct cyst have been observed in the liver. E. multilocidaris has also been found in the brain and lung (Ziegler). Hydatid rash. — When the fluid from an echinococcus cyst escapes into the peritoneal cavity it is apt to produce an urticarial eruption known as the hydatid rash. It usually appears shortly after the cyst has been ruptured or punctured ; it itches intensely, lasts two or three days, and is usually accompanied by high temperature and sometimes by abdo- minal pain. It is referred to by several observers. Krabbe writes : " A curious phenomenon is habitually observed when hydatids rupture into the peritoneal cavity : it provokes a transient urticaria." Finsen refers to two cases worth mentioning in rela- tion to the rash. Paul Helgason, aged 12 years, had for four years a large tumour in the right hypochondrium extending to the umbilicus. The lad received a blow upon the belly from a cow's horn that caused the tumour to disappear. Almost immediately the body was covered with a rash like an urticaria, but it soon disappeared. In another instance, a pregnant woman had a hepatic hydatid for six years. Three days after delivery, Avhilst lying quietly in bed, she was suddenly seized with acute pain in the abdomen ; the tumour of the liver disappeared, and in a short time the skin presented a papular rash. Hepatic hydatids may be accidentally ruptured in a variety of ways — such as blows, falls on the belly, by the wheels of a cart, or during an embrace in " a moment of exuberant affec- tion " (Treves). The usual mode of termination of an echinococcus cyst is that it ceases growing ; it then dies, shrivels up, and calci- fies, assuming a friable appearance like old mortar. When the cyst continues to grow its tendency is to rupture ; the great tension exerted by the accumulating fluid, and especially the formation of daughter-cysts, induces necrosis of portions of the cyst-wall. When contiguous to hollow viscera, such as the intestine, stomach, trachea, and 656 HYDATID CYSTS the like, the cyst is apt to come into contact with them, and the mutual pressure leads to absorption of the intervening tissue, and allows of the transmission of gas or air, or the osmosis of fluids which kill the parasite, and the entrance of pathogenic micro-organisms establishes suppuration. In many cases the communications between the colonies and the hollow viscera are so free that the contents are evacuated. In some instances this is a fortunate termina- tion ; but frequently it is a catastrophe to be dreaded, as it leads to secondary changes that have ultimately a fatal issue. In rare cases the vesicles in a colony become converted into colloid material of about the consistence of gelatme. Geographically, echinococcus disease has a very wide distribution, which corresponds with that of the dog. It is, however, far more frequent in some regions of the world than in others, especiall}^ where sheep-raising is a prime industry. Iceland is notorious for the frequency with which its inhabitants fall victims to this parasite ; after allowing great latitude for errors in the direction of excess in calculating its frequency, echinococcus disease must be regarded in the light of a persistent epidemic so far as that island is concerned. Next to Iceland, Silesia is usually regarded as the most infested district in Europe. In Australia this disease is excessively frequent, and whereas most of the monographs on this disease in its clinical aspects, written thirty years ago, were founded in a large measure on observations made in Iceland, we now look to the writings of Australian physicians and surgeons for information on the pathology, diagnosis, and treatment of echinococcus colonies. In Asia the disease is known : it occurs in India, though it is far from common. In America it is not frequent ; judging from the few references to it in American literature, hydatids appear to be far rarer in North America than in the British Isles. Zoologically, echinococcus disease is not very restricted, for it has been observed in monkeys, lemurs, cows, sheep, goats, deer, camels, antelopes, giraffes, ■ horses, asses, zebras, hogs, squirrels, and kangaroos, in addition to man. Topographical distribution in man. — Although an echino- CORPOREAL DISTRIBUTION 657 coccus cyst may form in almost any organ in the human body, it occurs with greater frequency in some organs and tissues than in others. A comparison of statistical tables compiled in Iceland, Germany, Australia, and America brings out most decisively the fact that hydatids are met with more frequently in the liver than in all other parts of the body together ; whilst in other organs, such as the breast, thyroid gland, or spinal cord, the literature of a century would furnish probably under a score of trustworthy cases. It is necessary to point out, in regard to the distribution of echinococcus colonies, that though on superficial examina- tion they may appear to be lodged in the liver, kidney, uterus, or rectum, a closer and more critical inquiry shows that in nearly all instances the parasite selects the loose subserous tissue. For example, echinococcus cysts in the liver usually lie in the tissue beneath the peritoneum covering this organ. This is certainly true of the uterus, and a few cases reported as growing from the Fallopian tube or ovary are really cases of infection of the loose connective tissue of the mesometrium. In the case of the kidney the parasite flourishes in the con- nective tissue of the renal sinus. This peculiar preference of the embryo of T. echinococcus for subserous areolar tissue will be further considered in de- scribing the relationship of the cysts and colonies in various organs. Echinococcus cysts may occur singly or be dis- tributed over the body in great numbers. The effects to which they give rise vary with the situation and dimensions of the cyst. For instance, a cyst of such a size as to cause no inconvenience when seated in the liver would, if growing in the brain or in the walls of the heart, induce death from mechani- cal causes. Again, a colony in the Hver will often attain a very large size before causing inconvenience to the patient, whereas one only half the size situated in the pelvis will produce much distress by interfering with the function of the rectum or bladder. On the other hand, a small cyst in the liver, no larger than an orange, when accidentally ruptured so that its contents escape into the peritoneal cavity, may rapidly destroy hfe, but a cyst the size of a melon, or larger, bursting into the rectum, will not lead to much trouble ; though even a small cyst so seated as to rupture into the trachea will, 2 Q 658 HYDATID GYSTS when the event comes to pass, almost by suffocation. Indeed, the ways m colonies kill are so many and so var dealt Avith under each organ. The bursting of a colony into the general infection of the peritoneum ; grafting themselves on this membrane like miliary tuberculosis. inevitably cause death Avhich these cysts and ious that they will be abdomen may lead to the brood-capsules en- produce an appearance Fig. 3-52. — Portion of liver in which the interlobular tissue throughout the organ was infested with echinococcus cysts. {Mtiseuin, Jtoyal College of Surgeons.) Liver. — Echinococcus cysts and colonies, as we have seen, are most frequent in the liver. This is not due to any selective power on the part of the parasite, but to the fact that it finds its way into the gastric tributaries of the portal vein, and is passively conveyed into the gland. As a rule, a single cyst is found in the liver, though it is not uncommon to find three or four ; but there is no limit to their number, and the museum of St. Thomas's Hospital contains a liver weighing nearly twenty-five pounds, obtained from a sailor HEPATIC HYDATIDS 659 in 1864, which is occupied by hundreds of cysts. The case was carefully described by Peacock. There were cysts in the lungs, spleen, kidney, omentum, and right ventricle of the heart. A portion of the liver is in the museum of the Royal College of Surgeons, and is the source of Fig. 352. A critical examination of the distribution of the cysts and colonies in the liver demonstrates that their primary seat is in nearly all instances the connective tissue immediately be- neath its peritoneal investment or in the portal fissures. In the very exceptional cases where the cysts are uniformly dis- tributed through the organ, as in Peacock's sailor, the six- hooked embryo has selected the interlobular connective tissue. Leuckart's feeding experiments throw a good light on this specimen. His greatest success occurred with the pig, which he says " may be very readily infected by the eggs of Tcenia echinococcus," and he points out that " it is remark- able that the cysts were all thickly distributed under the serous covering of the liver, and that upon both the concave and convex surfaces." Leuckart also clearly notices the relation of this parasite to the connective tissue of the liver, for he distinctly states in more than one place in his book that these early cysts were " everywhere in direct continuity with the connective- tissue trabecular network of the liver.'' And he writes : " In all cases, moreover, it was the interlobu- lar tissue that contained the parasites." This supports the teaching of Naunyn, that the embryos are distributed by the vascular system. In the liver of the sailor we have an example of infec- tion exceptionally severe, in which the parasites occupied the interlobular connective tissue of the organ as well as the subserous tissue. The relative frequency of these cysts in the liver, the large size they attain in this organ, and the risk they occasion to life have caused them to be very attentively studied. When the cyst ruptures spontaneously it may take various directions. Thus, it may burst into the pleura and give rise to fatal pleurisy. Should the lung be adherent to the dia- phragm, the cyst may open into it and the contents be discharged through the bronchial tubes and trachea. Under these conditions gangrene of the lung may follow the rupture. 660 HYDATID GYSTS In a few instances the cyst has burst into the pericardium. Such an accident is rapidly fatal, as the inundation of the peri- cardial cavity with fluid and vesicles embarrasses the heart. In some cases death has followed from pericarditis. Rupture of a large cyst into the peritoneal cavity leads to serious consequences, but even when the cyst is small it may lead to general infection of the peritoneum. In a case under my care there was reason to believe that a hepatic colony had ruptured into the lesser bag of the peritoneum, for the whole of the small omentum was thickly beset with little vesicles. Graham records a similar observation. A cyst has been known to rupture into the stomach, the vesicles being after- wards vomited ; and some have burst into the intestine, the contents of the cysts being discharged by the anus. Among the rarer directions, hydatids have been known to rupture into the biliary passages, the obstruction caused by the vesicles has induced jaundice, and their subsequent passage along the common duct has produced biliary colic. This is a serious complication and often terminates fatally. In several cases which have been carefully investigated the colony opened into the hepatic duct. The museum of the Middlesex Hospital contains a specimen illustrating this, and the common bile-duct is sufficiently dilated to admit an index finger. The patient was under the care of Murchison. Another excessively rare direction is for the cyst to rup- ture into the inferior vena cava, the contents reaching the right side of the heart. Cases have been reported in which the pressure of a cyst has induced atrophy of the intercostals, and its contents have been discharged externally. Cysts have also been known to burst externally near the umbilicus. Suppurating cysts may terminate in any of the directions mentioned above. Hepatic hydatids may cause death by their size embar- rassing respiration ; or by pressure on important organs, such as the vena cava, producing anasarca; or by hindering the circulation through the vena portse and causing ascites ; whilst suppuration will lead to exhaustion or induce death by septicaemia or pyaemia. Heart. — Echinococcus cysts and vesicles are met with in the heart under two conditions : (1) the cyst arises in the GAUD I AG HYDATIDS 661 loose areolar tissue of the organ, and is then termed "pri- mary " ; or (2) the vesicles are conveyed into the cavities of the right side of the heart as emboli in consequence of the bursting of a colony into some large efl'erent vessel like the vena cava. In most descriptions of " hydatids of the heart " attention is in the main directed to the relation of the cysts and colo- nies to the chambers of this organ, but a critical examination of the reports and specimens serves to show that the parasite exhibits the same fondness for abiding in loose areolar tissue in this organ as in others. The heart contains in the auriculo-ventrieular groove a large amount of loose adipose tissue which is strictly sub- serous. This loose tissue, which serves as a bed for the coronary vessels, penetrates deeply between the adjacent walls of the auricles, and indicates on the ventricular sur- face of the heart the line of the interventricular septum. A critical examination of some of the available specimens makes it clear that in the majority of instances the parasite lodges in the loose tissue of the auriculo-ventrieular septum. A man aged 19 years died in Guy's Hospital with extreme suffering and the ordinary symptoms of mitral imperfection. On examining the heart, Moxon found a projection the size of an apple on the back of the auricles, " off their septum near where it joins the septum of the ventricles ; from its extent it implicated all those parts mentioned." It had completely blocked the coronary sinus. The cyst, which con- tained daughter-vesicles, was unbroken (Fig. 353). A study of this specimen shows that the colony arose in the loose tissue of the auriculo-ventrieular groove and came into close relation with the four cardiac cavities. It is a noteworthy fact that the cyst is in very intimate rela- tion with the interventricular septum. I have come across several records in which the cyst is described as occupying this septum, and, on examining the specimen described by Peacock, which is preserved in the museum of the Royal College of Surgeons, the cyst will be seen to occupy its upper (auricular) end. The effects of echinococcus colonies on the heart and circulation are important. A cyst may exist for a long time 662 HYDATID GYSTS and give no indication of its presence, and then death occurs suddenl}' and the cause is manifest at the post-mortem examination (Peacock's case). In others the cyst, or colony, embarrasses the action of the heart and produces serious symptoms of valvular lesion (Evans, Moxon). More often the cyst bursts into one of the cavities of the heart, the vesicles and membrane being deported as emboli. When the cavities on the left side of the heart are invaded the vesicles Fig. 353. — Left ventricle of heart opened vertically to expose an echinococcus colony growing in the loose tissue of the auriculo-ventricular septum on the posterior aspect of the heart. {3£useum of the Middlesex Hospital.) are distributed by the systemic vessels. Oesterlin recorded a case in w^hich a girl of 23 years developed gangrene of the right leg ; this was amputated, and she died of pyaemia. An echinococcus colony the size of a pigeon's Qgg, situated in the wall of the left auricle, had burst into the cavity of the auricle, a piece of the cyst- wall was discovered in a thrombus in the right common iliac artery, and an entire vesicle had lodged in the deep femoral artery. A.ltmann has recorded a case which illustrates the tragic way in which an echinococcus colony of the heart may PULMONARY HYDATIDS 663 destroy life. A servant-girl was gathering chips at a wood- heap ; she fell down as if in a fit, and died within ten minutes. On post-mortem examination an echinococcus colony as big as an orange was found on the posterior aspect of the left auricle ; it had ruptured into the auricular cavity. A daughter -cyst had been conveyed into the left internal carotid artery and blocked it at its entrance into the cranium. A complete examination was not permitted. When a " colony " bursts into one or other cavity on the dextral side of the heart the vesicles and fragments of mem- brane are carried as emboli into the lungs (Budd, Barclay). Echinococcus cysts seated in the tissues of the heart are said to be primary, but the vesicles and membrane of a colony may find their way into the heart as emboli. This, however, is a very rare phenomenon, and after a careful search I can only refer to one — the classical observation reported by Luschke to Professor Leuckart. A woman 45 years of age died suddenly. In the posterior border of the liver there was an echinococcus cyst about the size of a child's head, which had burst through the walls and discharged some of its contents into the inferior vena cava. The daughter-cysts had reached the right chamber of the heart, and had been driven thence into the pulmonary arteries and caused rapid death. The most impressive feature connected with the clinical side of echinococcus colonies in the heart is the dramatic suddenness with which they may cause death, but this is no novelty in connexion with grave cardiac disorders of any kind. Lungs. — Echinococcus cysts occur in the lungs under two conditions. (1) The cyst — for it is usually single — may be situated wholly within the substance of the lung, and in most cases chooses the lower lobe, especially of the right lung ; or (2) it may grow in the tissue immediately beneath the pulmonary pleura and project as an outgrowth from the lung into the pleural cavity. When the cysts are small they occasion little inconvenience, but increasing in size they compress the lung and lead to hsemoptysis. Apart from the mere pressure effects produced by the cyst, it is liable to rupture into the bronchial tubes ; pieces 664 HYDATID CJY8T8 of membrane and vesicles are coughed up, and indicate the nature of the case. When the cyst communicates with a bronchial tube, suppuration of the cyst is the inevitable con- sequence. Should the cyst rupture into the pleural cavity, empyema is the usual result. It is well to bear in mind that, when vesicles and mem- brane are coughed up, it does not necessarily follow that the cyst is seated in the lung. Hepatic cysts are sometimes evacuated by this route. Kidney, Brood- capsules. Ureter. Mother-cyst. Fibrous capsule. Fig. 3.14. — Echinococcus colony occupying the sinus of the kidney. {Museum, Middlesex Hospital.) Kidney. — Echinococcus disease of this organ has often been recorded. The colony may lodge in the loose areolar tissue of the renal sinus (Fig. 354), or grow immediately beneath the capsule. In each situation it may attain a very large size and lead to extensive atrophy of the renal tissue. When of small size the cysts rarely give rise to trouble or even inconvenience during life, and their presence is onl}^ revealed in the course of a post-mortem examination. There are good reasons for believing that an echinococcus colony of the kidney may rupture into the pelvis of the organ, the fluid and vesicles passing down the ureter, to be PANCBEATia HYDATIDS 665 discharged by the urethra. This is, of course, the most satis- factor}'- mode of termination, except perhaps death of the parasite with subsequent calcilication. In the case of the right kidney, when the cyst- wall calcifies, it may form close adhesion to the walls of the in- ferior vena cava, and make it extremely dangerous to stri]^ the capsule from the vein. In at least one instance the vein has been torn in the process, with a fatal result. Pancreas. — An echinococcus colony of the pancreas is extremely rare. In an example under my care the colony was opened, emptied, and drained, as its enucleation was impracticable. The patient, a woman, died four weeks later from hsemorrhage due to ulceration of the inferior j)an- creatico -duodenal artery. Post-mortem, the dissection estab- lished the fact that the colony occupied the head of the pancreas. Thyroid gland. — Echinococcus cysts are rare in this situation ; they have been known to cause death by bursting into the trachea. Subperitoneal tissue and omenta. — These are extremely favourable situations in which the parasite can flourish, especially the great omentum, mesentery, mesocolon, and the connective tissue of the pelvis. Echinococcus colonies in the pelvis of women sometimes complicate pregnancy and obstruct delivery (Kllstner, Andrews, Blacker). Birch-Hirschfeld reported an instance of an echinococcus cj^st in the cavity of the vermiform appendix, which was dilated to twice the thickness of the thumb. It contained the remains of membrane which presented under the micro- scope the characteristic lamination. The appendix contained a great number of semitransparent vesicles, varying from a pin's head to a pea in size : most of these were sterile. The communication between the appendix and the ciecum Avas obliterated. The walls of the appendix and its mucous mem- brane were atrophied from the pressure exerted by the cyst, and presented mosaic-Uke marks caused by the pressure of the vesicles. The patient was a man 38 years of age. Scrotum. — A man supposed to have a hydrocele as big as an emu's egg was tapped by Moloney : it was an echinococcus cyst. 666 SYDATID GY8T8 Connective tissue of the trunk and limbs. — Many cases have been recorded in which echinococcus cysts have been found in the axilla, orbit, posterior triang'le of the neck, etc. Their nature is rarely suspected until the swelling is incised. Mamma. — Echinococcus cysts in this gland are very rare ; records of about twenty cases are accessible. The patients were in nearly all instances adult women. The disease takes the form of a slowly increasing, painless swelling, which may involve the whole breast or project as U teTU s Pe.riioiieum Eel 1 1 Fig. 355. — Echinococcus colonies in the mesometrium. {After Freuncl.) a smooth, elastic, fluctuating tumour from some portion of its circumference. These cysts may exist in the breast for ten years or longer without producing much inconvenience ; they have been reported with a capacity of twenty ounces. Occasionally the cyst suppurates. Diagnosis in countries where the echinococcus is not common is very difficult with- out the assistance of an exploratory puncture. Drawings of echinococcus colonies of the mamma are given by Astley Cooper, Bryant, and others. Uterus. — Echinococcus colonies have on several occasions been observed growing beneath the peritoneal investment of VT^RINE HYDATIDS 667 the uterus and forming a tumour as large as the patient's head (Fig. 355). Cysts of this character chnically simulate ovarian and uterine tumours, especially subserous fibroids (Altormyan). In one remarkable instance an echinococcus cyst 11 cm. in diameter grew beneath the serous covering of the fundus of the uterus and opened into the right Fallopian tube, which was greatly distended, thrown into con- volutions, and filled with vesicles (Moloney). Freund has published an admirable report of some examples of pelvic TUBE VARY Fig. 356. — A mesosalpinx with the tube and ovary in transverse section. The ovary is flattened upon the wall of an echinococcus colony occupying the mesosalpinx. * The cut surface of the Fallopian tube. hydatids ; it is the best contribution to the literature of this subject. His unique experience is probably due to the cir- cumstance that his observations were made in Silesia — a European region second only to Iceland in the frequency with which the inhabitants become infected by echinococcus. It is exceptional to find vesicles in the Fallopian tubes, but in a woman 32 years of age Doleris found them so stuffed with vesicles that they formed a large tumour reaching above the umbilicus. The mass weighed 2 kilogrammes, and consisted of the two tubes coiled upon themselves like small intestines, and so elons^ated that one measured 57 and the other 53 cm. 668 HYDATID CYSTS Ovary. — Neisser collected seven records of supposed hyda- tid cysts of the ovary, but an exammation of the original re- ports shows that there was little reason in most of the cases to class them with hydatids ; indeed, one of the cases was an ordinary multilocular ovarian cyst. Even in the examples re- corded in recent years, now that the term " hydatid " is almost restricted to the true echinococcus cyst, the cases recorded as " hydatid of the ovary " are conditions where the colony has grown primaril}^ in the mesometrium, and implicated the ovary secondarily. The specimen represented in Fig. 856 was removed by the author from a woman 44 years of age. It was as large as a turkey's egg, and freely movable in the belly. The colony arose in the mesosalpinx, and flattened out the ovary. As far as could be ascertained at the operation, there were no other cysts in the abdomen. Testis. — Echinococcus colonies have been seen in the scrotum, but I have never found any record of one within the tunica albuginea. ■ Brain. — Echinococcus cysts occur in connexion with the membranes of the brain ; the loose tissue of the pia mater is favourable to their growth. They are more frequent in re- lation with the cerebrum than the cerebellum. The pressure of such cysts produces a bay in the cortex of the cerebrum. It is often remarked by those who have recorded examples of intracranial hydatids that the damage produced by the cyst on the brain is out of proportion to the symptoms ; but the same is equally true of almost all cerebral tumours. Hydatid cj^sts of the brain are nearly always sterile, and they are not furnished with the thick fibrous capsule which surrounds them in the liver and omentum. Bones. — Echinococcus colonies are very rare in bones, and they seem to prefer the medullary cavities of long bones. The variety found in the bones of man is that known as Echino- coccus multilocular is, in which there is no mother-cyst, but the medullary cavity of the bone is occupied by a multitude of vesicles (Fig. 357). The effect of these colonies on the bone is very extraordinary ; they induce atrophy of its shaft, and at length the bone breaks from some trivial injury. In some instances operations have been undertaken for the relief Fig. 357. — Echinococ- cus multilocularis iu the shaft of tlie humerus ; from a woman 35 years of age who suffered amputation through the shoulder- joint. {After Graham.) Fig. 358. — Remnants of a femur and tibia fenestrated by a colony of Echinococciis multilocularis. {Museum of the Royal College of Surgeons.') 6G9 670 HYDATID CYSTS of abscesses supposed to be due to necrosis, and wlien the bone has been opened up vesicles have escaped. When the colon}^ occupies the end of a bone the vesicles may invade the adjacent joint (Fig. 358). Spine. — Echinococcus cysts occur in connexion with the spine under three conditions. 1. The cysts are situated entirely ivithin the canal. Such are divisible into two sets: (a) cysts lying inside the dural sheath, and (b) those which grow in the loose areolar tissue between the bone and the dura mater (Fig. 359). The ma- jority belong to the latter division. Schlesenger tabulated the variety and position of four Dura Mater -Cord Fig. 359. — Extradural eclimococcus cyst compressing the sx^iial cord at the level of the third cervical vertebra. (Modified from Colman.) hundred tumours of the spinal canal : forty-four were echino- coccus cysts, five were intradural and thirty-nine extradural. 2. The cysts arise in a vertebra and extend into the neural canal. Primary echinococcus colonies of the vertebrae are examples of E. onultilocularis (Fig. 360). 3. Echinococcus colonies groiving in tissues adjacent to the spine inay involve the vertebrcc and extend into the spinal canal. Symptoms and diagnosis. — The localizing symptoms depend entirely on the situation of the cyst. For example, when the cyst or colony is in the spinal canal the symptoms DIAGNOSIS 671 will be those common to any tumour large enough to com- press the spinal cord and produce paraplegia. In the cranial cavity the symptoms are identical with those produced by any tumour which compresses the brain. In the abdomen, especially when the cysts are connected with the liver, the nature of the swelling may be suspected when it is painless and slowly increasing, and especially if a peculiar vibratory thrill is produced by percussing the middle finger of the left hand when it is laid firmly over the tumour. When the colonies burst into hollow viscera and the characteristic vesicles are dis- charged by the bowel, urethra, vagina, trachea, or through suppurating sinuses, then the nature of the disease is self- evident. In countries where hydatid disease is endemic, it is usual, in cases in which a tumour or swelling exhibits negative characters, to regard it as an echinocoecus cyst. More than half the examples of this disease occur as sur- prises in the course of surgical operations. To aid diagnosis, efforts have been made by Welch and Chapman to ob- tain a precipitin reaction Treatment. — This is al- ways surgical, and the parti- cular method of carrying it out varies with the situation ot the cyst. When they hang as big as apples from the omentum, it is only necessary to expose the cysts through an incision in the belly-wall, ligature the pedicle, and remove them. In many cases they are firmly adherent to surrounding struc- tures. In these circumstances the fibrous capsule should be freely incised and the mother-cyst enucleated ; the empty Fig 360. — Echinocoecus muUilocularin in the seventh cervical vertebra. [Museum of St. George'' s Sospitcd.^ {After Bennett.) 672 HYDATID CYSTS capsule rarely gives trouble. Suppurating cysts demand free incision and drainage. In removing colonies it is wise to avoid soiling the edges of the wound with brood-capsules, as they may give rise to cysts of some size in the cicatrix. In the case of the liver, echinococcus colonies treated by incision, enucleation of the capsule, and free drainage give but .little trouble. Great care should be taken thoroughly to remove the mother-cyst, for decomposition of this tissue when left is a source of grave danger. All meddling methods — as punctures with trocars, aspiration, and electrolysis — cannot be too strongly condemned. Echinococcus cysts in the lungs require to be treated on the principles of empyema, and in this situation Lendon particularly insists on the necessity of removing the mother- cyst. The contents of dead colonies are sometimes so firm that they require removal with a scoop. When the cyst-wall is calcified it often leads to a persistent sinus. In the case of bones the treatment consists of incision, evacuation of vesicles, and drainage. Exceptionally, when the bone is seriously damaged, fractured, or a large joint is invaded, amputation has been found a necessity. Echinococcus cysts within the cranium have been local- ized, exposed by trephining, and, after evacuating the fluid, the cyst has been successfully extracted (Yerco, Rennie and Crago, Mills and McCormick). Echinococcus cysts in the spinal canal have been suc- cessfully submitted to surgery (Tytler and Williamson). The difficulty of localizing and. treating some of these cases is well shown by a case recorded by Stewart McKay. Single echinococcus cysts and colonies give, as a rule, admirable results when treated surgically, but in cases where the patient suffers from a general infection the disease is very inveterate, demands much persistence on the part of the surgeon, and calls for great courage and fortitude on the part of the patient. In a woman with general dissemination of hydatids in the omentum and subperitoneal tissue, I removed numerous large echinococcus colonies on seven occasions in nineteen years. She remains in apparently good health. Between the second and third operations she successfully conceived. HYDATID CYSTS 673 Altmann, " Hydatid of Heart ; rupture into left auricle and plugging of left carotid artery." — Intercolonial Med. Journ. of Australia, 1902, p. 573. Andrews, R. H., " A Case in which Pregnancy was complicated by the presence of a Hydatid Cyst in the Pelvis."— /c»?/r«. of Vhstet. and Gyn., 1908, xiv. 333. Barclay, J., "A Case of Hydatids of the Heart and l^wag^"— Glasgow Med. Journ., N.S., 1867, i. 426. Bryant, T., " Diseases of the Breast," 1887, Plate viii., Figs. 3 and 4. Budd, W., "Hydatid Tumour in Apex of Right Ventricle of the Heart."— Trans. Path. Soc, 1859, x. 80. Colman, W. S., "Hydatid Cyst of the Spinal Canal, causing Paraplegia."— St. Thomas's Hasp. Repts., 1899, xxviii. 361. Cooper, Sir Astley, " Diseases of the Breast," 1829, Plate ix. Evans, H. R., "Case in which a Cyst containing Hydatids was found in the substance of the Heart." — Med.-Chir. Trans., 1832, xvii. 507. Finsen, Jon, " Les Echinocoques en Islande." — Arch. Gen. de Med., 1869, xiii. 23. Freund, W. A., " Gynakologische Klinik," Strasburg, 1885, i. 203. Graham, James, "Hydatid Disease," 1891, pp. 134, 137. Krabbe, " Recherches Helminthologiques en Danemark et en Islande," 1866. Klistner, " Kaiserschnitt wegen eines Echinokokkus imBecken." — Zentralbl. f. Gyn., 1907, xxxi. 1390. Lendon, A. A., " Clinical Lectures on Hydatid Disease of the Lungs," 1902. Leuckart, R, " The Parasites of Man," 1886. McKay, W. J. Stewart, Australian Med. Gaz., Feb. 20, 1906. Mills and McCormick, Australian Med. Journ., Nov. 1904. Moloney, " Hydatid of Pelvis opening into the Right Fallopian Tube."— Australian Med. Journ., 1879, i. 478. Moxon, W., " Hydatid of the Heart obliterating by its pressure the Coronary Sinus."— I'raws. Path. Soc, 1869-70, xxi. 99. Muskett, P. E., " Unusual Site for Hydatid Cyst; an addition to the recognized varieties of intrascrotal disease." — Australasian Med. Gaz., 1886-7, vi. 57. Oesterlin, Otto, "Ueber Echinococcus an Herzen." — Virchow's Arch, f.path. Anat., 1868, xlii. 404. Peacock, T. B., and Wale Hicks, J., " Hydatids in Liver, Spleen, Right Kidney, Omentum, Lungs, and Heart." — Trans. Path. Soc, 1863-64, xv. 247. Rennie and Crago, Australian Med. Gaz., 1902, p. 547. Schlesenger, Beit. z. Klinik der Riockenmark und Wirhelttimoren, Jena, 1898. Treves, Sir Frederick, " Peculiar Mode of Rupture of a Hydatid Cyst." — Trans. Clin. Soc, 1887-88, xxi. 82. Tytler, P., and Williamson, R. P., " Spinal Hydatid Cysts causing severe Compression Myelitis." — Brit. Med. Journ., 1903, i. 301. 2 R 674 HYDATID CYSTS Verco, Dr., "Intercolonial Medical Congress of Australasia: Section of Surgery ; Discussion on Hydatids." — Brit. Med. Journ., 1892, ii. 1066. Virchow, Rudolph, " Die multiloculare ulcerirende Echinokokkengeschwulst der Leber." — Verh. d.phys. med. Ges. z. Wiirzhurg, 1856, vi. 84. Virchow, Rudolph, " On Multilocular Hydatid." — Australian Med. Jouroi., 1884, p. 171. Welch, D. A., and Chapman. H. G., "The Precipitin Reaction in Hydatid Disease."— Zawce^, 1908, i. 1338, and 1909, i. 1103. Ziegler, E., " Tasnia Echinococcus ]\Iultilocularis." — Lehrh. d. allg. Path. u. path. Anat., 1905, i. 762. INDEX Aoardiacs, 428 Accessary adrenals, 102 thyroids, 472 . tragus, 484 Adenoeele, 248, 252 Adenoma, 248 cystic, 248, 252 fibro-, 250 of breast, 248, 250 of intestine, 249 • of liver, 345 of ovary, 488, 494, 504 of palate, 92 of pituitary body, 317 of stomach, 249 of testis, 539 of thyroid, 249, 313 ■ of uterus, 373 sebaceous, 309 Adeno-myoma, 375 Adeno-sareoma, 248 Adrenal tumours, 106 ■ in adults, 109 in children, 106 Adrenals, accessary, 102 Age-distribution of tumours, 9 Age-incidence of fibroids, 195 Albinism, 110 Alimentary canal, sarcoma of, 72 Alkaptonuria, 114, 120 AUantois, cysts of, 599 Amorphous acardiacs, 428 Ampulla of Vater, cancer of, 354 Anaplasia, 284 Aneurysm by anastomosis, 159 cirsoid, 159 Angeioma, 156 cavernous, 157 of brain, 161 of breast, 157 of conjunctiva, 157 of heart, 158 of labium, 157 of larynx, 158 of lip, 157 of liver, 159 of muscles, 158 of subperitoneal tissue, 159 of synovial membrane, 159 of tongue, 158 plexiform, 159 simple, 156 Angeio-sarcoma, 410 Anidian monsters, 428 Antenatal hydronephrosis, 577 Antrum, carcinoma of, 325 Anus, carcinoma of, 343 Appendices epiploicse, 16 Appendix {see Vermiform appendix) Archoplasmic vesicles, 282 Atrophic cancer, 260 Auricles, cervical, 477 sebaceous cysts of, 484 Auricular dermoids, 481, 483 fistulse, 481, 482 Autosite, 422 Axial rotation of fibroids, 193 of ovarian tumours, 529 Bartholin's gland, carcinoma of, 370 Bile-ducts, carcinoma of, 353 Biologic theory of cancer, 281 Bird's-eye inclusions in carcinoma, 282 Bistournage, 532 Bladder, carcinoma of, 363 diverticula of, 612 myoma of, 59 sacculated, 612 villous papilloma of, 238 Blood-supply of sarcomas, 62 Bone, carcinoma (secondary) of, 269 chondroma of, 26 hydatid cysts of, 668 lipoma of, 21 myeloma of, 45 osteoma of, 34, 37 sarcoma of, 77 Brain, angeioma of, 161 glioma of, 148 hydatid cysts of, 668 • neuroma of, 131 Branchial fistula, 474 Brawny arm in carcinoma of breast, 297 Breast, adenoeele of, 248 adenoma of, 248, 250 angeioma of, 157 carcinoma of, 287, 292 cysts of, 253, 303 duct-carcinoma of, 303 duct-papilloma of, 303 ■ endothelioma of, 410 hydatid cysts of, 666 675 676 INDEX Breast, sarcoma of, 75 Bronchocele, 313 Brood-capsules, 653 Bunion, 625 Burrowing tendencies of sarcoma, 66 Bursse, adventitious, 624 sarcoma of, 71 subtendinous, 624 Caecum, carcinoma of, 337 Calcification of lipomas, 14 of sarcomas, 69 Calculus of salivary glands, 570 Cancer [see Carcinoma) Canceroderms, 119 Carcinoma (cancer), acinous (of breast), 292 arising in innocent tumours, 10 atrophic, 260 biologic theory of, 281 causes of, 275 colloid, 259, 332 contact-transference, 272 countryman's, 319 degeneration of, 259, 332 dissemination of, 263 duct-, 303 emboli in, 266 embryonic theory of, 275 en cuirasse, 296 gall-stones in relation to, 349 gland-, 258 gland-infection in, 260 ■ heredity in, 273 • infection of, 270 inoperable, 287, 291 lymphatics of, 260 melano-, 117 of ampulla of Vater, 354 of antrum, 325 of anus, 343 of Bartholin's gland, 370 of bile-ducts, 353 of bladder, 363 of bone, 269 of breast, 287, 292 of caecum, 337 • • of cheek, 322 ■ of clitoris, 369 of conjunctiva, 256 of Cowper's gland, 367 of Fallopian tube, 399 of fauces, 322 of gall-bladder, 348 of gum, 322 ■ — — of hepatic duct, 353 of intestine, 336 of jaw, 322 Carcinoma (cancer) of kidney, 361 of labium, 368 of larynx, 7, 325 of lip, 319 of liver, 346 of lung (secondary), 266 of oesophagus, 328 of ovary, 522 (secondary), 524 of palate, 322 of pancreas, 355 • of penis, 366 of pharynx, 322 of piima, 256 of prostate, 269, 365 of rectum, 336, 342 of scars, 256 of scrotum, 365 of sebaceous glands, 311 of stomach, 330 of testis, 537 ■ of thyroid, 315 of tongue, 321 of tonsil, 322 of ureter, 363 of urethra, 364 of uterus, body, 389 with fibroids, 395 neck, 380 with fibroids, 393 of vagina, 369 of vermiform appendix, 337 of vulva, 368 oophorectomy in, 280 parasitic theory of, 278 parenchyma of, 255 peritoneal infection in, permeation in, 267 recurrence of, 301 rodent, 311 squamous-celled, 255 stroma of, 255 sweep's, 255 terminal infections in, theory of, biologic, 281 embryonic, 275 parasitic, 278 trauma in relation to, treatment of, 287 varieties of, 254 withering, 260 X-ray, 257 Cartilage-containing tumours, 26, 55, 546 Cartilaginous tumours (see Chon- droma) Cavernous lymphangeioma, 163 naevus, 157 Cementoma, 215 265 268 277 INDEX 677 Cervical fibroids, 173 fistulae, lateral, 474 median, 468 teeth, 559 Cervix, erosion of, 373 Chsetodon, 41 Cheek, cancer of, 322 Chloroma, 121 Chondroma, 26 calcification of, 26 of bone, 26 of joints, 29 of larynx, 30 of nose, 30 of pelvis, 28 ossification of, 26 Chorion-epithelioma, 9, 415 benignum, 417 in teratoma, 420 malignum, 419 Choroid plexus, papilloma of, 241 Chromatophores, 112 Chyle-cysts, 609 Cicatrix, carcinoma of, 256 horns of, 242, 245 Ciliated epithelium in ovarian cysts, 491 Cirsoid aneurysm, 159 Classification of tumours, 4 Clavicle, sarcoma of, 86 Clitoris, carcinoma of, 369 Coccygeal sinus, 444 Colloid degeneration in carcinoma, 259, 332 Colon, carcinoma of, 341 lipoma of, 18 • teratoma of, 437 Composite odontoma, 222 Compound follicular odontoma, 216 Conglomerate fibroids, 169 Conjoined twins, 422 Conjunctiva, angeioma of, 157 carcinoma of, 256 dermoids of, 127 lipoma of, 17 moles of, 127 Connective-tissue tumoiirs, 12 Corneal cysts, 464 Corpus luteum, 507 Cowper's gland, carcinoma of, 367 Cryptophthalmos, 129 Cutaneous horns, 242 Cystic adenoma, 248, 252 disease of testis, 538 Cysts, allantoic, 599 bursal, 624 chyle-, 609 dental, 230 dentigerous, 212 Cysts, dermoid (of ovary), 492 Gartnerian, 518 hydatid {see Hydatid cysts) implantation-, 462 involution-, 303 lutein (see Lutein cysts) Mullerian, 600 neural, 629 of breast, 253, 303 of cornea, 464 of eyeball, 573 of eyelid (dacryops), 573 of finger, 462 of gall-bladder, 569 of hyaloid canal, 573 of iris, 464 of joints, 620 of kidney (congenital), 586 of lachrymal gland, 573 ■ of liver, 564 multiple, 565 single, 566 of neck, 164, 476 of ovary, 488, 491, 494, 504, 535 of pancreas, 571 of pharynx, 612 of salivary glands, 570 of testicle, 538 of thyroid gland, 314 of umbilicus, 594 of urachus, 599 of vermiform appendix, 564 oophoronic, 487 papillomatous, of breast, 303 of ovary, 509 of thyroid, 314 parovarian, 514 pseudo-, 611 retention-, 563 sebaceous, 307 synovial, 620 teno-synovial, 622 vitello-intestinal, 594 Cytological transformations of malig- nant tumours, 281 Dacryops, 573 De Morgan spots, 119 Deeiduoma, 415 Degeneration-changes in carcinoma, 259 in fibroids, 199 in sarcoma, 69 Demodex folliculorum, 307 Dental cysts, 230 Dentigerous cysts, 212 Dermatitis, X-ray, 257 678 INDEX Dermoid pterygium, 127 Dermoids, auricular, 483 of back, 442 of conjunctiva, 127 of dura mater, 459 of face, 448, 455 of inguinal canal, 446 of labium, 446 of lung, 434 of neck, 447, 468 of nose, 457 of orbito-nasal fissure, 455 of ovary, 487, 492, 495, 535 of pinna, 483 of rectum, 437 of sacrum, 429 of scalp, 459 of scrotum, 445 of sternum, 447 of testicle, 445, 542 of thorax, 434, 447 of tongue, 467 sequestration-, 442 • tubulo-, 442 Dichotomy, 424 Digits, bursse of, 625 chondroma of, 26 cysts of, 462 lipoma of, 13 melanoma of, 115 Dissemination of carcinoma, 263 of sarcoma, 63 Distribution of sarcoma, 69 Diverticula, intestinal, 611 laryngeal, 618 oesophageal, 615 pharyngeal, 612 synovial, 622 tracheal, 615 vesical, 612 Duct of Gartner, 487, 515 cysts of, 518 Dura-endothelioma, 410 Dura mater, dermoids of, 459 • endothelioma of, 410 Ecchondrosis, articular, 29 larjmgeal, 29 nasal, 30 Echinococcus disease (see Hydatid cysts) Echinococcus mailtilocularis, 654 Egg-shell crackling, 48 Embryoma, malignant, 505 ovarian, 487, 492, 502 testicular, 542 Embryonal rudiment in ovarian dermoids, 503 Embryonal rudiment in teratoma of testis, 542 Embryonic theory of cancer, 275 Endothelioma, 405 of breast, 409 of dura mater, 410 of gum, 405 of omentum, 410 of ovary, 523 • of salivary glands, 405 • of skin, 410 of uterus, 410 Environment in relation to tumours, 4 Epignathus, 436 Epithelial balls, 497 infection in ovarian embryomas, 505 odontoma, 211 Epoophoron, 487 Erosion of cervix, 373 Eustachian pouches, 617 Evolution of central nervous system, 649 Exostoses, 37 from inflammatory products, 41 subungual, 41 Eyeball, carcinoma of, 117 cysts (of cornea) of, 464 (of iris) of, 464 glioma of, 151 melanoma of, 116 Eyelid, coloboma of, 128 cysts (dacryops) of, 573 dermoids of, 455 nsevi of, 157 Face, angeioma of, 156 dermoids of, 448, 455 mandibular tubercle of, 451 ■ moles of, 126 Fallopian tube, carcinoma of, 399 • papilloma of, 398 pseudo-cysts of, 627 Fatty hernise of linea alba, 16 tumour (see Lipoma) Fauces, carcinoma of, 322 Feet, sarcoma of, 88 Femur, periosteal sarcoma of, 78 Fibro-adenoma of breast, 250 Fibro-cystic tumour, 180 Fibroids, age-incidence of, 195 axial rotation of, 193 carcinoma of, 393, 395 changes (secondary) in, 180 clinical characters of, 206 conglomerate, 169 degeneration of, 199 extrusion of, 189 INDEX 679 Fibroids, growth of, 186 impaction of, 192 incarceration of, 192 interstitial, 169 intestinal obstruction by, 193 intracervical, 173 intramural, 168, 192 latent, 172 lymphatics in, 185 malignant changes in, 184 menopause and, 203 menstruation and, 195 necrobiosis of, 183 of cervix uteri, 173, 192 (intracervical), 173, 190 of malformed uterus, 178 of mesometrium, 176 of ovarian ligament, 177 of ovary, 520 of round ligament, 177 of utero -sacral ligament, 178 pregnancy with, 198 rate of growth of, 186 red degeneration of, 183, 201 secondary changes in, 180 septic infection of, 188, 199 structure of, 180 submucous, 169 subserous, 171, 192 treatment of, 207 tubal pregnancy with, 203 uterine, 168 Fibro-myoma, 168, 181 Fibrous odontoma, 215 Fistula, auricular, 482 branchial, 474 cervical, 468, 474 • coccygeal, 444 nasal, 457 Fleshy j)olypus of womb, 170 Follicular odontoma, 212 Functionless ducts, 594 Galaetocele, 253 Gall-bladder, carcinoma of, 348 cystic, 569 mucocele of, 569 Gall-stones, cancer and, 349 Ganglion, compound, 623 simple, 622 Ganglionic neuroma, 131 Gartner's duct, 487, 515 cysts of, 518 Gastric lipoma, 17 General thyroid malignancy, 104, 316 Gland-cancer, 258 Glandular organs, sarcoma of, 95 Glioma, 134, 148 of brain, 148 of retina, 151 of spinal cord, 153 Gliomatous disease, 9 Gonads, 550 Goundou, 41 Gum, carcinoma of, 322 endothelioma of, 405 sarcoma of, 90 Guttural pouches of horses, 616 Haematocele of tunica vaginalis, 603 Haemendothelioma, 405 Hsemorrhage with fibroids, 187 Hand, lipoma of, 13 sarcoma of, 88 Heart, angeioma of, 168 hydatids of, 660 lipoma of, 21 Hepatic duct, carcinoma of, 353 Heredity in cancer, 273 Hernial lipoma, 16 Heterotopic teeth, 553 Hibernating gland, 20 Hodgkin's disease, 53 Homogentisinic acid, 120 Horned men of Ivory Coast, 40 Horns, cicatrix-, 242, 245 cutaneous, 242, 244 in ovarian dermoids, 496 nail-, 242, 246 sebaceous, 242 treatment of, 246 wart-, 242, 244 Hyaloid canal, cysts of, 573 Hydatid cysts, 652 of bone, 668 of brain, 668 ■- of breast, 666 of connective tissue of limbs and trunk, 666 of heart, 660 of kidney, 664 of liver, 658 of lungs, 663 of omentum, 665 of ovary, 668 of pancreas, 665 of scrotum, 665 • of spine, 670 of testicle, 668 of thyroid, 665 of uterus, 666 of vermiform appendix, 665 disease, geographical distribu- tion of, 656 rash of, 655 680 INDEX Hydatid disease, symptoms and diagnosis of, 670 treatment of, 671 zoological distribution of, 656 mole, 417 Hydramnion, 629 Hydrocele, congenital, 605 encysted, of cord, 603 of testis, 550 funicular, 606 of canal of Nuck, 606 of fourth ventricle, 634 of hernial sac, 606 of tunica vaginalis, 602 omental, 609 ovarian, 607 ■ rupture of, 605 treatment of, 606 Hydrocephalus, 629, 646 Hydrocystoma, 238 Hydrometra, 564 Hydronephroma, 102 Hydronephrosis, 564, 575 antenatal, 577 bilateral, 575 ■ ■ intermitting, 581 unilateral, 579 Hydroperitoneum, 511 Hydrosalpinx, 609 Hypernephroma, 102 Hypertrichosis, 645 Ileum, imperforate, 597 septate, 597 Impaction of fibroids, 192 Imperforate hymen, 578 ileum, 597 pharynx, 613 ■ urethra, 578, 589, 591 Implantation-cysts, 462 Inadequacy of ureter, 582 Infant Hercules, 107 Infective granuloma, 1 Infiltrating properties of sarcoma, 64 Inguinal canal, dermoids of, 446 Innominate bone, sarcoma of, 87 Inoperable cancer and its treatment, 287, 291 Interdigital pouch, 445 Intermandibular fissure, 452 Intermuscular lipoma, 19 Interstitial fibroids, 169 intestinal obstruction with, 193 Intestinal obstruction by fibroids, 193 Intestine, adenoma of, 249 carcinoma of, 336 Intestine, diverticula of, 61] lipoma of, 17 sarcoma of, 73 Intra-abdominal teratoma, 433 Intracervical fibroids, 173 Intracranial teratoma, 435 Intracystic warts, 238 Intradural lipoma, 22 Intramural fibroids, 168 Intramuscular lipoma, 21 Intra-ocular melanoma, 116 Intrathoracic teratoma, 434 Involution -cysts of breast, 303 Iris, cysts of, 464 melanoma of, 116 Jaundice in carcinoma of pancreas, 357 Jaw, carcinoma of, 322 osteoma of, 34, 39 ■ sarcoma of, 89 Joints, chondroma of, 29 cysts of, 620 lipoma of, 18 loose bodies of, 29, 30 Kidney, carcinoma of, 361 congenital cystic, 586 hydatids of, 664 hydronephrosis of, 564, 675 papilloma of, 239 rotation of, 579 sacculated, 584 sarcoma of, 96, 101 Kobelt's tubes, 515 Kraurosis vulvae, 368 Labium, angeioma of, 157 carcinoma of, 368 dermoids of, 446 lipoma of, 16 Lachrymal gland, dacryops of, 573 Langhans' layer, 415 Laryngocele, 618 Larynx, angeioma of, 158 carcinoma of, 7, 325 extrinsic, 326 intrinsic, 325 chondroma of, 30 diverticula of, 618 lipoma of, 5, 17 • neuroma of, 146 papilloma (wart) of, 237 Latent fibroids, 172 Leio-myoma, 60 Leucoderma, 1 10 Leukaemia, 53 INDEX 681 Leukoplakia, 321 Leukoplakic vulvitis, 368 Liability of different organs tumours, 3 Lip, angeioma of, 157 carcinoma of, 319 lipoma of, 17 Lipoma arborescens, 18 calcified, 14 . clinical features of, 23 hernial, 16 intermuscular, 19 intradural, 22 intramuscular, 21 meningeal, 22 nasi, 138 of bones, 21 of broad ligament, 16 of colon, 18 of conjunctiva, 17 of fingers, 13 of foot, 13 of hand, 13 of heart, 21 of hernial sac, 16 of intestine, 17 of joints, 18 of labium, 16 of larynx, 5, 17 of lip, 17 of meninges, 22 of mesometrium, 16 of muscles, 19 . of neck, 14 of nerves, 22 of pa,lm, 13 of scalp, 13 of scrotum, 16 of sole, 13 of spermatic cord, 16 of spinal cord, 22 of stomach, 17 of tongue, 19 parosteal, 21 subcutaneous, 12 submucous, 17 subperitoneal, 16 subpleural, 17 subserous, 15 subsynovial, 18 treatment of, 24 Liver, adenoma of, 345 angeioma of, 159 carcinoma of, 346 cysts of, 564 hydatids of, 658 Loose bodies in hydroceles, 605 in joints, 29, 30 cartilages, 29, 30 I Lung, carcinoma (secondary) of, 266 dermoids of, 434 to I hydatids of, 663 Lutein cysts, 507 in relation to hydatid mole, 417 Lymphadenoma, 53 Lymphangeioma, 162 cavernous, 163 lingual, 162 Lymphatic cyst, 164 nsevus, 162 Lymphatics of carcinoma, 260 of fibroids, 185 Lymph-gland infection in carcinoma, 260 Lympho-sarcoma, 53, 547 Macroglossia, 143, 162 Macrostoma, 451 Malignant transformation of fibroids, 184 of tumours, 10 tumours, cytologic transforma- tions of, 281 inoperable, 287 relation of trauma to, 277 Mamma- (see Breast) Mandible, osteoma of, 34 sarcoma of, 89 Mandibular recesses, 451 tubercles, 451 Masked spina bifida, 644 Mastoid teeth, 556 Maxilla, osteoma of, 40 sarcoma of, 89 Meckel's diverticulum, 596 Median cervical fistula, 468 Melanin, 113 Melanism, 110 Melano-carcinoma, 117 Melanoma, 6, 112 of eyeball, 116 Melanosis, 110 Melanotic sarcoma, 112 Melon-seed bodies, 622, 623 Meningeal lipoma, 22, 649 Meningocele, 634, 641 Meningo-encephalocele, 634 Meningo-myelocele, 640 Menopause in fibroids, 203 Menstruation and fibroids, 195 Mesentery, chyle-cysts of, 609 lipoma of, 16 Mesometrium, fibroids of, 176 lipoma of, 16 Mesonephros, 514, 537, 588 Metaplasia, 284 682 INDEX Microstoma, 451 Moles, 114, 123 conjunctival, 127 Molluseum fibrosum, 134, 136 Mouse-tumours, malignant, 285 Mucocele, 569 Miillerian ducts, cysts of, 600 Multilocular cystic epithelial tumours, 212 cysts of ovary, 488 Multiple neuromas, 134, 146 Muscles, angeioma of, 158 lipoma of, 19 sarcoma of, 70 Myelocele, 639 Myeloid sarcoma, 45 Myeloma, 9, 45 clinical characters of, 47 treatment of, 48 Myoma, 59 ■ of alimentary canal, 59 of bladder, 59 uterine, 168 Myo-sareoma, 55 Myosotis ossificans, 41 Myxoma, 58 Myxo-sarcoma of omentum, 332 Naevo-lipoma, 14 Naevus (see Angeioma) pilosus, 123 spUosus, 114, 123 Nail-horns, 242, 246 Nasal chondroma, 30 dermoids, 457 polypi, 59 Naso-pharyngeal tumours, 89 Neck, auricles of, 477 • cysts of, 164, 476 dermoids of, 447, 468 fistulas of, 468, 474 lipoma of, 14 sebaceous cysts of, 476 teratoma of, 561 Necrobiosis of fibroids, 183 Necrosis of ovarian tumours, 532 of sarcoma, 69 Nerves, lipoma of, 22 neuroma of, 130, 143, 144, 146 — — sarcoma of, 70, 134 Neural cysts, 629 Neuro-fibromatosis, 134 Neuro-lipoma, 22 Neuroma, 130 ganglionic, 131 of facial nerve, 143 of hypoglossal nerve, 143 of laryngeal nerve, 146 Neuroma of lingual nerve, 143 of musculo-spiral nerve, 144 of occipital nerve, 143 of trigeminal nerve, 143 plexiform, 134, 140 Nose (see Nasal) Nuck, hydrocele of canal of, 606 Obsolete canals, 466, 594 Ochronosis, 120 Odontoma, 211 clinical characters of, 228 composite, 222 compound follicular, 216 epithelial, 211 fibrous, 215 follicular, 212 radicular, 218 treatment of, 230 (Esophagus, carcinoma of, 328 diverticula of, 615 myoma of, 59 Omentum, endothelioma of, 410 hydatid cysts of, 665 hydrocele of, 609 in cancer of stomach, 332 Oophoron, 486 cysts of, 487 Operculum, 478 Optic nerve, sarcoma of, 150 Orbito-nasal fissure, dermoids of, 455 Osseous tumours, 34 Ossification of tendons at attachment, 38 Osteoma, 34 cancellous, 37 clinical characters of, 41 compact, 34 facial, 34, 39 of auditory meatus, 36 of frontal sinus, 35 of mandible, 34 of maxilla, 40 of orbit, 35 treatment of, 42 Ovarian adenoma, 488, 494, 504 carcinoma, 524 cysts, multilocular, 488, 494, 504 rupture of, 535 papillomatous, 509 simple, 488 — — with ciliated epithe- lium, 491 dermoids, 492, 495, 502 suppurating, 535 embryoma, 492, 495, 502 — — malignant, 505 endothelioma, 523 INDEX 683 Ovarian fibroids, 520 hydatids, 668 hydrocele, 607 ligament, fibroids of, 177 mammse, 498 papillomatous cysts, 509 sarcoma, 522 teeth, 553 teratoma, 492, 495, 502 • tumour (solid), 8 axial rotation of, 529 necrosis of, 532 Pachydermatocele, 138 Painful subcutaneous tubercle, 130 Palate, adenoma of, 92 carcinoma of, 322 sarcoma of, 92 teratoma of, 435 Pancreas, carcinoma of, 355 cysts of, 571 hydatids of, 665 Papilloma, 235 cutaneous, 235 intracystic, 238 of bladder, 238 of choroid plexus, 241 of endometrium, 375 of Fallopian tube, 398 of larynx, 237 of ovarian cysts, 509 of renal pelvis, 239 of uterus, 375 villous, 238 Parasitic foetus, 422, 544 theory of cancer, 278 Parathyroid bodies, 316 tumours, 6 Paroophoron, 486 Parovarian cysts, 514 Parovarium (epoophoron), 487 Patella, sarcoma of, 88 Pelvis, chondroma of, 28 Penis, carcinoma of, 366 ■ torsion of, 578 warts of, 235 Perirenal sarcoma, 101 Perithelioma, 405, 410 Peritoneal infection in carcinoma, 265 Permeation in carcinoma, 267 Pharyngocele, 612 Pliarynx, carcinoma of, 322 cysts of, 612 diverticula of, 612 imperforate, 613 pouches of, 612, 614 teratoma of, 435 Pigmented nodes, 114 Pigmented tumoiirs, 110 warts, 115 Pinna, dermoids of, 483 fistulse of, 482 sebaceous cysts of, 484 Pituitary body, 317 Plexiform angeioma, 159 neuroma, 134, 140 Plimmer's bodies, 282 Polypi, nasal, 59 uterine, 374 Port-wine stains, 156 Postanal dimple, 444 gut, 437 Postrectal teratoma, 438 Pouchj interdigital, 445 pharyngeal, 612, 614 Rathke s, 612 Precancerous conditions, 258 Pregnancy and carcinoma of cervix uteri, 388 and fibroids, 198 Prostate, carcinoma of, 269, 365 sarcoma of, 95 Psammoma, 410 of cerebral membranes, 410 of spinal membranes, 413 Pseudo-cysts, 611 connected with Fallopian tube, 627 Pyometra, 383, 564 Pyonephrosis, 564, 585 Racemose sarcoma of cervix uteri, 56 Radicular odontoma, 218 Radium treatment of carcinoma, 288 of naevi, 161 of rodent ulcer, 312 Radius, sarcoma of, 86 Ranula, 570 pancreatic, 571 Rectum, adenoma of, 249 carcinoma of, 336, 342 dermoids of, 437 polypi of,- 249 teratoma of, 437, 440 Recurring fibroids, 58 Red degeneration of fibroids, 183, 201 Renal {see Kidney) " Rests " in carcinoma, 275 Retained testis, malignant disease of, 548 Rete mirabile, 186 Retention-cysts, 563 Retina, glioma of, 151 Retroperitoneal sarcoma, 57 Rhabdo-myoma, 55 Ribs, sarcoma of, 88 684 INDEX Rodent ulcer, 311 Round-celled sarcoma, 52 Round ligament, fibroids of, 177 Rudiment, embryonic, in ovarian dermoids, 503 Sacro-coccygeal tumour, 438 Sacrum, dermoids of, 429 Salivary glands, calculus of, 570 chondrifying tumour of, 405 cysts of, 570 endothelioma of, 405 mixed tumour of, 405 sarcoma of, 405 Sarcoma, 52 adeno-, 248 angeio-, 410 blood-supply of, 62 burrowing tendencies of, 66 calcification of, 69 degeneration of, 69 dissemination of, 63 distribution of, 69 grape-like (racemose), 56 — — histology of, 52 infiltration of, 64 lympho-, 53, 547 melanotic, 112 myo-, 55 of alimentary canal, 72 of bone, 77 of breast, 75 of bursas, 71 of feet, 88 of femur, 78 of gum, 90 of hands, 88 of intestine, 73 of jaw, 89 of kidney, 96, 101 of mamma, 75 of miiscles, 70 of nasal septum, 89 of naso-pharynx, 89 of nerves, 70, 134 of optic nerve, 150 of ovary, 522 of paired viscera, 100 of palate, 92 of patella, 88 of prostate, 95 of radius, 86 of ribs, 88 of salivary glands, 405 of scapula, 86 of skull, 88 of sternum, 88 Sarcoma of stomach, 73 of synovial membrane, 70 of testicle, 547 of tibia, 83 — " — of ulna, 86 of uterus, 56 of vagina, 74 of vermiform appendix, 73 of vertebrae, 92 perirenal, 101 racemose, 56 relation of, to veins, 67 retroperitoneal, 57 round-celled, 52 secondary changes in, 69 deposits of, 63 spindle-celled, 53 subperitoneal, 57 treatment of, 75 Scalp, dermoids of, 459 horns of, 243 lipoma of, 13 sebaceous cysts of, 307 Scapula, sarcoma of, 86 Scars of burns, carcinoma of, 256 Scolices, 653 Scrotum, carcinoma of, 365 dermoids of, 445 horns of, 243 hydatids of, 665 lipoma of, 16 warts of, 255 Sebaceous adenoma, 309 cysts, 307 of pinna, 484 glands, carcinoma of, 311 horns, 242 Secondary carcinoma of liver, 347 • of lung, 266 changes in fibroids, 180 in sarcoma, 69 deposits of carcinoma, 263 of sarcoma, 63 Septate ileum, 597 Sinus (see Fistula) Skin, endothelioma of, 410 of ovarian dermoids, 496 Skull, sarcoma of, 88 Solid ovarian tumours, 8 Soot-wart, 256 Spermatocele, 550 Spina bifida, 638 complications of, 646 masked, 644 occulta, 644 treatment of, 648 with cranial meningocele, 636 Spinal cord, glioma of, 153 INDEX 685 Spinal cord, lipoma of, 22 Spindle-celled sarcoma, 53 Spine, dermoids of, 443 hydatids of, 670 Spontaneous fracture, 296 Squamous-celled carcinoma, 255 Sternum, dermoids of, 447 sarcoma of, 88 Stomach, adenoma of, 249 carcinoma of, 330 leio-myoma of, 60 lipoma of, 17 myoma of, 59 sarcoma of, 73 Structure of tumours, 2 Struma suprarenalis, 103 Subconjunctival lipoma, 17 Subcutaneous lipoma, 12 Submucous fibroids, 169 lipoma, 17 Subperitoneal angeioma, 159 lipoma, 16 nsevus, 159 sarcoma, 67 Subpleural lipoma, 17 Subserous fibroids, 171, 192 lipoma, 15 Subsynovial lipoma, 18 Subungual exostoses, 41 Sucking-cushion, 19 Sulcus, naso-facial, 457 Supracondyloid process of humerus, 39 Sweep's cancer, 255, 365 Syncytium, 415 Synovial angeioma, 159 cysts, 620 sarcoma, 70 Syrlngo-myelocele, 640 Tails, 650 Teeth, cervical, in sheep, 559 heterotopic, 553 mastoid, 556 ovarian, 495, 553 tympanic, 556 Telangeiectasis, 156 Tendons, ossification of,, at attach- ments, 38 Teratoma, 422 chorion-epithelioma and, 420 ■ external, 422 internal, 433 intra-abdominal, 433 intracranial, 435 intrathoracic, 434 of colon, 437 of neck, 561 Teratoma of palate, 435 of pharynx, 435 of testis, 542 ovarian, 492, 495, 502 postrectal, 438 rectal, 437, 440 Testicle, adenoma of, 539 carcinoma of, 537 cystic disease of, 538 dermoids of, 542 encysted hydrocele of, 550 ■ hydatid cysts of, 668 malignant disease in retained, 548 myo-sarcoma (rhabdo-myoma) of, 55 sarcoma of, 547 teratoma of, 542 Thorax, dermoids of, 434, 447 Thyro-glossal duct, 466 Thyroid, accessary, 472 adenoma of, 249, 313 carcinoma of, 315 cysts of, 314 gland in ovarian dermoid, 499 hydatids of, 665 malignancy, general, 104, 316 papillomatous cysts of, 314 pyramidal lobe of, 472 Tibia, sarcoma of, 83 Tongue, angeioma of, 158 carcinoma of, 321 • dermoids of, 467 ichthyosis of, 321 lipoma of, 19 lymphangeioma of, 162 lympho-sarcoma of, 53 Tonsil, carcinoma of, 322 Torsion of ovarian tumours, 529 of penis, 578 Toxin treatment of malignant disease, 289 Tracheal diverticula, 615 Tragus, accessary, 484 Transformation of innocent into malig- nant tumours, 10 Treatment of tumours (general), 11 Tubal pregnancy with fibroids, 203 Twisted pedicle of fibroids, 193 of ovarian tumours, 529 Ulcer, rodent, 311 Ulna, sarcoma of, 86 Umbilicus, cysts of, 594 tumours of, 594 Undescended testis, malignant disease in, 548 Urachus, cysts of, 599 686 INDEX Ureter, carcinoma of, 363 inadequacy of, 582 Urethra, carcinoma of, 364 imperforate, 578, 589, 591 Utero-sacral ligament, fibroids of, 178 Uterus, adenoma of, 373 adeno-myoma of, 375 carcinoma of body of, 389 with fibroids, 395 of neck of, 380 with fibroids, 393 chorion-epithelioma benignum of, 417 malignum of, 419 endothelioma of, 410 fibroids of, 168 hydatids of, 666 myoma of, 168 papilloma of, 375 sarcoma of, 56 Vagina, carcinoma of, 369 — — cysts of, 518 sarcoma of, 74 Veins, relation of, to sarcoma, 67 Vermiform appendix, carcinoma of, 337 cysts of, 564 Vermiform appendix, hydatids of, 665 sarcoma of, 73 Vertebrae, sarcoma of, 92 Villous papilloma, 238 of bladder, 238 of choroid plexus, 241 of kidney, 239 Vitello-intestinal duct, cysts of, 594 Vulva, carcinoma of, 368 Wandering goitre, 315 Wart-horns, 242, 244 Warts, 234 • intracystic, 238 laryngeal, 237 of penis, 235 soot-, 256 Warty ovaries, 514 Wens, 307, 459 Withering cancer, 260, 293 Wolffian body, 514, 537, 588 Woolner's tip, 485 Xanthoma, 121 X-ray carcinoma, 257 • dermatitis, 257 treatment, 288 Printed by Cassell and Company, Limited, La Belle Sauvage, London, B.C. COLUMBIA UNIVERSITY LIBRARIES This book is due on the date indicated below, or at the expiration of a definite period after the date of borrowing, as provided by the library rules or by special arrangement with the Librarian in charge. DATE BORROWED DATE DUE DATE BORROWED DATE DUE m 1^ iUSi. . j 1 1 » C2a(94e)MIOO \: ^ it / COLUMBIA UNIVERSITY LIBRARIES (hsi.stx) RD 651 B61 1911 C.1 Tumours, innocent and malignant: 2002284499 KD651 B61 1911 Bland-Sutton ^ocent and malignant - RESERVE SHfLL-f X^P ^ ^l