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Cullen TS. Embryology, anatomy, and diseases of the umbilicus together with diseases of the urachus. (1916) W. B. Saunders Company, Philadelphia And London.

Umbilicus (1916): 1 Umbilical Region Embryology | 2 Umbilical Region Anatomy | 3 Umbilical New-born Infections | 4 Umbilical Hemorrhage | 5 Umbilicus Granuloma | 6 Omphalomesenteric Duct Remnants | 7 Umbilicus Abnormalities | 8 Meckel's Diverticulum | 9 Intestinal Cysts | 10 Patent Omphalomesenteric Duct 1 | 11 Patent Omphalomesenteric Duct 2 | 12 Bowel Prolapsus at Patent Omphalomesenteric Duct | 13 Abdominal Wall Cysts by Omphalomesenteric Duct Remnants | 14 Omphalomesenteric Vessels Persistence | 15 Umbilical Inflammatory Changes | 16 Subumbilical Space Abscess | 17 Umbilicus Paget's Disease | 18 Umbilicus Infections | 19 Umbilicus Abnormalities 2 | 20 Umbilicus Fecal Fistula | 21 Umbilicus Round Worms | 22 Umbilicus Foreign Substance Escape | 23 Umbilical Tumors | 24 Umbilicus Adenomyoma | 25 Umbilicus Carcinoma | 26 Umbilicus Sarcoma | 27 Umbilical Hernia | 28 The Urachus | 29 Congenital Patent Urachus | 30 Urachus Remnants | 31 Urachal Remnants Producing Tumors | 32 Large Urachal Cysts | 33 Anterior Abdominal Wall Abscesses | 34 Urachal Cavities | 35 Umbilicus Acquired Urinary Fistula | 36 Urachal Concretions and Urinary Calculi | 37 Urachus Malignant Changes | 38 Urachus Bleeding into the Bladder | 39 Patent Urachus Tuberculosis | Figures
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Chapter XXIV. Adenomyoma of the Umbilicus

Historic sketch. Report of cases. Personal observations.

UMBILICAL TUMORS CONTAINING UTERINE MUCOSA OR REMNANTS OF MULLER'S DUCTS.*

While gathering together from the literature the numerous cases of primary tumor of the umbilicus I found several that did not seem to belong to any of the classes hitherto recognized, and yet all of these cases in one or more points bear a certain amount of resemblance to one another. Finally, the picture of this newgroup became so firmly fixed in my mind that when reading the description of a case recorded in 1899 by Dr. Green, of Romford, England, I felt so sure that his case came under this category that I wrote him, asking if perchance he still had a section of the tumor. An examination of the slide which he kindly furnished me showed that we were right in our surmise. In brief, the clinical histories in this class of cases, coupled with the gross appearances of the tumors, leave no doubt that we are dealing with a variety of umbilical tumor never before clearly understood.

The composite picture of such tumors — which were found only in women — is as follows: At some time between the thirtieth and fifty-fifth year a small tumor develops at the umbilicus, reaching its full size in the course of a few months. It is usually described as being the size of a small nut. Sometimes it is painful, especially at the menstrual period, and in at least one instance there was a brownish, bloody discharge from the umbilicus at such times.

The overlying skin is usually pigmented, and there may be one or two bluish or brownish cysts just beneath the skin. These may rupture and discharge a little brownish fluid— old blood. On section the nodule is found to be intimately attached to the skin, is very dense, and is traversed by glistening bands of fibrous tissue. Scattered throughout the nodule one sometimes finds small spaces presenting a sieve-like appearance. These spaces are filled with brownish fluid. Occasionally there may be a small cyst, several millimeters in diameter, filled with

  • Shortly after the appearance, in Surgery, Gynecology and Obstetrics (May, 1912, 479), of

my article on Umbilical Tumors Containing Uterine Mucosa or Remnants of Miiller's Duct, I received the following, in a letter from Dr. S. W. Goddard, of Brockton, Mass., dated September 10, 1912: "After reading your recent article in Surgery, Gynecology and Obstetrics on Umbilical Tumors and noting a similarity to two I have published, I am sending you a reprint of the same in hopes that they may be of interest to you, and, if of any value, would be glad to have you make use of them in connection with your work, as I infer that you are specially interested in the subject. I have not seen any similar cases since."

These two cases reported by Dr. Goddard belong to the same group as those I have collected. That he clearly recognized the source of origin of these glands is also evident from the title of his article: Two Umbilical Tumors of Probable Uterine Origin. I had overlooked Dr. Goddard's article completely. To him undoubtedly belongs the credit for having drawn attention to the probable origin of the glands in these cases. Dr. Goddard's cases, one recently recorded by Barker, and one examined by me for Dr. Jones, of Atlanta, are recorded at the end of the chapter.

373


374 THE UMBILICUS AND ITS DISEASES.

brownish contents. Exceptionally, grayish, somewhat homogeneous areas are distinguishable in the tumor.

On histologic examination the superficial squamous epithelium is usually found intact. It may be normal or thickened. The stroma of the growth is composed of dense fibrous tissue. Sometimes a few bundles of non-striped muscle are noted here and there in the fibrous stroma. In other specimens the non-striped muscle is much more abundant than the fibrous tissue.

Scattered throughout the field are glands, round, oval, or irregular. They occur singly or in groups, and are lined with cylindric epithelium. When occurring singly, they frequently lie in direct contact with the fibrous tissue, but when found in groups, are usually surrounded by a characteristic stroma that stains more deeply and is much more cellular than the surrounding fibrous tissue. The cells of this stroma between the glands usually have oval or round vesicular nuclei. Frequently some of the glands are dilated and their epithelium is somewhat flattened. The cyst spaces, noted macroscopically and filled with brownish fluid, are likewise dilated glands, and the fluid is old blood. The stroma around the glands frequently shows fresh hemorrhage or remnants of old blood, to be recognized by the deposit of blood pigment.

From the above description it is clearly seen that the gland picture is that of the uterine mucosa with its typical glands and its characteristic stroma, and further that the typical menstrual reaction is often present, as evidenced by the pain in the nodule at the periods, the accumulation of old menstrual blood with the formation of small cysts, and in at least one instance by the occasional discharge of blood from the umbilicus. In this case (Fig. 168) one or two of the glands opened directly on the surface, thus allowing free escape of the menstrual blood.

In all, nine cases have been recorded. Green's case (Fig. 168), Mintz's first and third cases (Figs. 171 and 174), and Ehrlich's case (Fig. 177) owe their glandular origin without doubt to the uterus or to a portion of Miiller's duct from which the uterine mucosa originally comes. Although the cases reported by Wullstein, Giannettasio, von Noorden, and Mintz (Case 2) also probably belong to the same group, the evidence is not quite so clear, and without the opportunity of carefully studying the original sections I should not feel justified in including them as certain instances.

The most common glandular elements at the umbilicus are remnants of the omphalomesenteric duct. These are usually identical in structure with the glands of the small intestine, and never give rise to the cystic dilatations noted in the group of cases under discussion; moreover, hemorrhage into the stroma is exceptional. They differ totally both in their gross and histologic appearances.

We have in this group of cases glandular elements that from their histologic appearance and arrangement correspond exactly with those found in adenomyoma of the uterus, and in one case at least (Green's) the surrounding stroma was composed chiefly of non-striped muscle, making the growth essentially an adenomyoma. In the majority of the cases, however, the stroma consisted of fibrous tissue, but little muscle being present.

These growths are benign, and if removed in toto, provided no other embryonic foci exist, give rise to no further trouble. In Mintz's first case, four years after the first nodule had been removed, two others developed. These were also extirpated.

In Ehrlich's case, in addition to typical uterine mucosa, there was a definite tumor formation that had originated from sweat-glands.


ADENOMYOMA OF THE UMBILICUS. 375

In order that the reader may gain a clear insight into each of the cases, they are reported in detail, together with the comments on each case.

The descriptions of the illustrations naturally differ from those given by the various authors. I have redescribed each picture in the light of our new knowledge of the subject.

A Small Umbilical Tumor Containing Uterine Glands.* — [The author very kindly placed a section of the growth at my disposal. There is no doubt that the gland elements in this case are identical with those of the uterine mucosa, as seen from Figs. 168, 169, and 170, which have recently been made. — T. S. C]

The patient, a woman fifty years of age, had complained of irritation about the umbilicus for about two and a half years, and there had been an occasional discharge, brownish in color. When Dr. Green saw her, fourteen months before the growth was removed, there was some eczematous irritation of the skin in the neighborhood, but no projecting growth could be observed at that time. The bottom of the umbilical depression had an irregular, wart-like appearance. The surrounding eczema soon yielded to treatment, but there was from time to time an irritating discharge from the umbilicus, which the patient declared was always worse during her menstrual periods.

The umbilicus with the growth and a portion of the surrounding skin was removed. The omentum was not adherent to the umbilicus, and no intestine was seen at operation. The wound healed by first intention and there was no subsequent trouble, so far as could be learned.

On microscopic examination the skin was found to be normal. The stroma of the growth was made up of fibrous tissue and non-striped muscle, scattered among which, without any definite arrangement, were numerous gland elements. Some of these were very near the free surface, others more deeply placed. They were for the most part tubular and lined with columnar epithelium showing large, deeply staining nuclei. They were thought to be reproductions of Lieberkiihn's crypts, but differed from them in their exaggerated dimensions. Some of them were so large that they might almost have been described as cysts. [Dr. Green thought that the growth was a remnant of the vitello-intestinal tract.]

On reading this history I noted that there had been some discharge of blood from the umbilicus, as indicated by the brownish color, and, furthermore, that the patient had always been worse at the menstrual periods. This made me suspect the possible presence of uterine glands at the umbilicus. I wrote Dr. Green and early in July received the following reply:

The Ferns, Romford, England, June 22, 1911. Dear Sir: In reply to your query about my case of umbilical growth, I am pleased to be able to send you a section from the same, so that you may form your own judgment as to its histology. I did not think it was malignant. I last heard of the patient two and a half years after the operation. She was then alive and well. This, I think, shows that the growth was not secondary to an undiagnosed growth within the abdomen. Owing to removal, I have not subsequently heard of her, so I cannot say what ultimately happened to her. I inclose a copy of my paper which I happened to have kept.

Yours faithfully,

Charles D. Green.

  • Green, Charles D. : A Case of Umbilical Papilloma Which Showed Some Activity of Growth

in a Patient Fifty Years of Age and Which was Due Apparently to Inclusion of a Portion of Meckel's Diverticulum. Trans. Path. Soc. London, 1899, 1, 243.


376


THE UMBILICUS AND ITS DISEASES.


We were particularly fortunate in obtaining this specimen from Dr. Green, in the first place, because it was twelve years since the case had been reported, and,



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Fig. 168. — A Small Umbilical Tumor Containing Glands and Stroma Identical with Those of the Uterine

Mucosa. The slide was kindly furnished me by Dr. Charles D. Green, of Romford, England, and is from the umbilical growth reported by him in the Transactions of the Pathological Society of London, 1899. The squamous epithelium is intact, and apart from some thickening appears normal. Scattered throughout the underlying stroma are oval, round, or irregular glands occurring singly or in groups; there are also a few cystic spaces. Some of the glands lie directly beneath the skin. At c two of the glands open directly upon the surface of the umbilicus. Area A has been enlarged and is shown in Fig. 169. The increased magnification of area B is seen in Fig. 170. The photomicrographs of this series were made by Mr. H. H. Hart.

in the second place, because it is one of the most valuable cases of this character thus far on record.

Dr. Green's specimen, No. 125. — The skin surface is intact and practically normal, although at a few points the epithelium is considerably thickened. In


ADENOMYOMA OF THE UMBILICUS.


377


one or two places directly beneath the skin there is small-round-cell infiltration, chiefly in foci. At one point the surface epithelium extends a short distance into a cavity (Fig. 168, c). In the lower portion of the cavity the lining consists of cylindric epithelium, one layer in thickness. Around this area the stroma shows a considerable amount of hemorrhage. It is from this point that there was undoubtedly bleeding at the menstrual periods. The underlying stroma consists to a large extent of non-striped muscle. Scattered here and there throughout the muscle



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Fig. 169. — Glands from a Small Umbilical Tumor. The picture is an enlargement of the area A in Fig. 168. The normal character of the surface epithelium is clearly seen. The gland spaces vary considerably in size and shape and are lined with cylindric epithelium. Those in the picture lie in direct contact with the dense surrounding stroma.


are glands. They are small, round, oblong, irregular, or large (Fig. 169). A few of them occur singly and lie in direct contact with the surrounding stroma. The majority, however, occur in groups or in chains, and are separated from the surrounding stroma by a definite stroma of their own (Fig. 170), which is recognized by its deeper stain and its abundance of vesicular nuclei, which are oval or round. Some of the glands are very much dilated. Where such dilatations have taken place the surrounding stroma frequently shows a good deal of hemorrhage.


378


THE UMBILICUS AND ITS DISEASES.


Were it not for the presence of the skin surface one would immediately diagnose the specimen as an adenomyoma of the uterus. The picture is typical, as seen from Figs. 168, 169, and 170. The growth is an adenomyoma of the umbilicus. Dr. Green at the time felt sure that the condition was a rare one, as indicated from a second communication dated August 4, 1911:

Dear Dr. Cullen: .... I am glad you found my specimen so interesting. I had some photographs prepared, but the Committee of the Pathological Society did not think them of sufficient interest to insert them in the Transactions. I was a little disappointed at the time, for I thought that the condition was uncommon.

Yours faithfully,

Charles D. Green.



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Fig. 170. — Typical Uterine Mucosa in a Small Umbilical Ttjmob. An Enlargement op Area B in Fig. 168. The three large glands in the right-hand part of the picture, in shape and arrangement, resemble those found in an adenomyoma of the uterus: separating them from the dense tumor growth is a definite and characteristic stroma. The group of glands in the middle of the picture is even more characteristic, one of the glands being dilated. All are lined with cylindric epithelium, and the contrast between the surrounding stroma and the dense growth is very clearly marked. Afl noted in the description, non-striped muscle was found scattered throughout the nodule.


[On looking up the Transactions, I found that two of the committee diagnosed the growth as a columnar-cell carcinoma, but whether primary or secondary they were unable to decide. The chairman of the committee said some of the members present who examined the specimen were not inclined to regard it as malignant. There is little wonder that at that time confusion existed, and had it not been for the specially favorable opportunity I had had of examining so many cases of adenomyoma, I should have undoubtedly overlooked the true origin. — T. S. C]


ADENOMYOMA OF THE UMBILICUS. 379

Adenomyoma of the Umbilicus.* — Case 1 . — In 1883 a woman acquired an umbilical hernia after labor. Ten years later, within the space of about two months, a dark-blue tumor the size of a hazelnut developed on the umbilical elevation. This had two cystic areas on its surface. During menstruation the tumor swelled and the cysts ruptured. They contained blood-tinged fluid. The tumor was extirpated and the hernia repaired. This tumor on section presented a cavernous appearance, but no microscopic examination was made. In 1897, four years later, there was a return of the hernia, and at the umbilicus were two hard nodules about the size of hazelnuts. On microscopic examination they were found to contain glands lined with cylindric epithelium and surrounded by a definite stroma. Here and there bundles of non-striped muscle were in evidence. The dilated glands contained blood-pigment. Mintz thought he was dealing with remains of the omphalomesenteric duct. -- "ishh^^h

[When discussing this case some three years ago, just after making the •':•••

abstract, I made the following note: "The clinical history, the macroscopic appearance, the picture of the glands, the stroma, and the contents of the dilated glands all point to acleno- )

myoma, although adenomyoma of the I ,

umbilicus has never been reported." — T. S. C]

We are fortunate in again hear- J ,,. r , „ TT

=> -blG. 171. (jr LANDS IN A SMALL I MB1LICAL TCMOR.

ing from Mintz on this subject. Ten (Mintz, Case i.)

years later he published an article The outl ying connective-tissue stroma is very ir ., , (it\ -y U 1 1 "AT regular. Occupying the lower half of the field are glands

entitled DaS JNabeladenom, Arch. showing some branching. They are lined with one layer

f. klin. Chil*. 1909 lxxxix 385. Here of cylindric epithelium and lie in a characteristic stroma

, . ., . i j. -i -i-1, l," which separates them from the fibrous tissue of the

he CieSCriDeS, more in detail, the hlS- tumor. The entire picture reminds one to a large extent

tologic findings Of the Same Case. He of adenomyoma of the uterus.

says :

' ' The ground substance of the growth consists of connective tissue not very rich in cells. They cross one another or run parallel with one another in cords. Here and there in the scar tissue one sees gland tubules in either transverse or longitudinal section. They are surrounded by young, very cellular connective tissue, which passes very gradually into the old scar tissue. The glands are lined with one layer of cylindric epithelium. Their lumina are collapsed and contain blood pigment or reddish-colored contents (Fig. 171). In some places the tubules lie close, at other points the} r are separated. The newly formed connective tissue surrounding them has changed into old connective tissue poor in cell nuclei. Some of the glands are dilated and their epithelium is flattened. The lumina appear to be filled with detritus. Here and there the cylindric epithelium is unrecognizable and the cavity contains blood-pigment (Fig. 172). W'here the dilatation has occurred, the epithelium has disappeared; in this way are to be explained the cysts with blood contents which were noted when the patient first entered the hospital. Between the glandular portion of the tumor there are at some points groups of non-striped

  • Mintz, W.: Das wahre Adenom des Xabels. Deutsche Zeitschr. f. Chir., 1899, li, 545.


380 THE UMBILICUS AND ITS DISEASES.

muscle-fibers that have no definite topographic arrangement in relation with the glands. The microscopic examination shows an adenomatous growth in the scar tissue. This has stimulated the growth of the scar tissue, and thus originated the young connective tissue surrounding the new glands. In the mean time the periphery of the nodule in the scar has been converted into sarcoma."

[After giving this description he says in a foot-note that at the time of writing (that is, ten years later) the tumor had not returned. The explanation of the origin of this tumor he gives as persistent remains of the omphalomesenteric duct which had remained latent for forty-two years in the umbilical scar, and under the influence of chronic injury (a ten-year persistent umbilical hernia) had given rise to adenoma.

It can hardly be doubted that we are dealing with an adenomyoma, although such a case had heretofore never been described. We have the increase in size at the menstrual period, the cysts with blood contents, glands resembling uterine glands, the characteristic stroma of the mucosa surrounding the glands, that was

thought by Mintz to be sarcomatous, and the fact that, after the second operation, the patient remained absolutely well for - - - U ten years. How these glands originated

at the umbilicus we do not attempt to ex\ plain. We have, however, found them in

the inguinal region, and I feel confident \ that, in the course of time, somebody will

"""-.. get a clear chain of evidence showing how

remnants of the uterus can reach the umbilicus.— T. S. C]

Fig. 172. — Dilated Glands in a Small Umbilical a a ™ „ 1 i TT m b i 1 i C 8 1 T U m O r

Tumor. (Mintz, Case 1.)

In the center of the field is a very much dilated C O n t a i 11 i 11 g U t e r 1 11 e G 1 a 11 d S .

gland. Its epithelium is flattened. The gland itself is C a S e 2 (Mintz) . The WOllian Was

separated from the surrounding stroma by a definite, , -, . , • i , r tt i '"•'" v . •.'•

was 3 cm. in diameter. Ma- v.. •'•>. 4'V : "--/ v v° /"■ ' ■ ' '".-^k. ■'.'■ "-.• f'4

croscopically, it consisted of a '* ..- V/ v v v~... ,'. . / .' . '

hard, pure white, scar-like tis- ■ ':'! ; •■+ '■'. ■ '■■'■•■. ■-■ ■. "."

sue firmly attached to the skin. ,'.'•. ' ' !-.;•'•. 'v ;

Scattered throughout the turn- m. '■' ■-•../ ..■•' .; "'■... \ .'..-.■

or were a number of pin-head- , v ••• .. . • , v..

sized spaces which contained a % . '." ' '• .< ' v

serosanguineous fluid. His- V. ' v . .. ; f .;1 : \ : ; ;•'•' . ,

tologically, the chief mass con- . /' ' '..';.'"•', KP >, .■■■.'

sisted of fibrous tissue, poor in ... • '•'.; - . . • .. "V- ( - '

nuclei and cell-elements. The OU ;'••-} • ..." "• :;,

skin covering the tumor, ex- ...■'"■ . ; . ..:-■.•

cept that it showed a marked - .-'- ^^/{uH

pigmentation of the basal layer, looked normal. The connective tissue of the skin passed directly into that of the underlying tumor. In the tumor were numerous islands of loose connective tissue which varied markedly in the number of their nuclei; and inside this were epithelial elements. There were two definite histologic pictures. In the portion lying near the skin (Fig. 176) were groups of closely compressed and tortuous gland loops lined with large cuboid epithelial cells having small, centrally located nuclei. The gland lumina and the basement membrane of the tubal glands were easily recognizable. Similar glands were also found in the connective tissue. They were undoubtedly hypertrophic sweat-glands.

Predominating in the central portion of the extirpated tumor was a second kind of epithelial tissue likewise situated in the loose connective tissue, but exceedingly rich in nuclei. This consisted of tubular glands with high cylindric epithelium; cilia and goblet-cells were not visible. Through the fork-like arrangement of the tubular glands there had originated here and there many bay-like spaces which might be mistaken for papillary formations and which had given rise to cystic formations due to the presence of fluid. Here and there the epithelium of the cystic


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Fig. 176. — A Tumor of the Umbilicus Composed Partly of Hypertrophic Sweat-glands. (After H. Ehrlich.) The glands are gathered into definite groups, reminding one of the gland arrangement in small fibromata of the breast. The individual glands bear a marked resemblance to ordinary sweat-glands. Some of them are dilated. Another portion of the tumor consisted of typical uterine mucosa (see Fig. 177).


384 THE UMBILICUS AND ITS DISEASES.

spaces had disappeared or become flattened. The contents of the cysts were hemorrhagic or showed a formless detritus, and in several places surrounding the cysts were masses of blood-pigment. Van Gieson's stain failed to bring out any smooth muscle surrounding the epithelial elements. This was found only in connection with the vessels of the connective tissue and there not abundantly.

While the glands first described are without doubt hypertrophic sweat-glands, the glands of the second group are, on account of their character and their epithelium, in all probability derivatives of the intestinal tract. Ehrlich speaks of the growth as an adenoma of the umbilicus.

[The reader will note that, judging from Fig. 176, there is no doubt that the first gland elements described by Ehrlich are sweat-glands and that the tumor consisted of sweat-glands. Fig. 177, however, shows everywhere, that the second variety of



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Fig. 177. — Uterine Mucosa in an Umbilical Tumor. (After H. Ehrlich.) To the left are characteristic uterine glands, a few of them dilated. They are surrounded by a definite stroma which separates them from the connective tissue. In the right portion of the picture are similar glands, the majority of which have become dilated. If we take the left half of the picture only, it might very readily pass without any description for a representation of an adenomyoma of the uterus.

glands can in no way be connected with remnants of the intestinal duct, but that we have here typical uterine mucosa enveloped in a definite stroma.

The cystic spaces, as noted in the text, were partly filled with blood. They are nothing more than glands that have been markedly dilated by old menstrual fluid. This is one of the cases in which the definite uterine character of the mucosa is clearly evident. — T. S. C]

A Tumor of the Umbilicus Consisting of a Cystadenoma of the Sweat-glands and a Cavernous Angioma. (Eine Geschwuht d e s N a b e 1 s . Kombination von Cystadenom d e r Schweissdrusen m i t cavernosem Angiom.) — Wullsfein* says that in the literature he has found no tumor similar to the one he is describing. In 1891 a specimen was sent to the Gottingen laboratory. This consisted of an umbilical tumor which had developed in the course of three years and

  • Wullstein, L.: Arb. a. d. Path. Inst, in Gottingen, R. Virchow, zum 50. Doctor-Jubilaum, 1893, 245.


ADENOMYOMA OF THE UMBILICUS. 385

was attached by a thin pedicle, which had not been completely removed. The pedicle had extended into the abdominal cavity. The physician in charge had made a diagnosis of myxofibroma. The patient was a sterile woman thirty-four years of age. In addition to the umbilical tumor, another growth was present in the pelvis. This was the size of a fist, was connected with the uterus, and had spread out diffusely in the neighborhood of the right broad ligament. It could not be regarded as an exudate. The physician was interested to find out whether there was any connection between the two tumors; in other words, whether the umbilical growth was a metastasis. Wullstein examined a Muller's fluid specimen. It was everywhere covered with skin. It had a semicircular form and was about the size of a thaler. The umbilicus was raised 1 cm. above the surrounding abdominal skin, and its surface showed numerous shallow furrows. The umbilical furrow was recognized as an irregular, transverse cleft, which divided the umbilicus into two unequal portions, it becoming deeper and deeper in the middle until there was a depression 11 mm. in depth. About the middle of the under surface of the tumor was a cord about 1 cm. long, hardly as thick as a straw. This was solid and composed of connective tissue. The tumor itself was about 3 cm. long and averaged 1.5 cm. in thickness. On section it was seen that the umbilicus was everywhere covered with skin, which in all portions was thickened and markedly pigmented. From the bottom of the umbilical depression and running parallel were thick bundles of dense connective tissue. The tumor consisted of numerous dense, hard, glistening connective-tissue bundles, which enclosed more or less long or round areas of loose tissue, grayish in appearance, and in the interior in places were small lumina. Subcutaneous fat was absent. In the vicinity of the umbilical scar the tissue was sieve-like. The spaces of the meshwork were filled with dark-brown masses about the size of poppy-seeds. The meshwork consisted of firm connective tissue.

Microscopic examination of a section from the middle of the tumor showed that the epidermis was thickened. The deepest cells of the stratum mucosum were granular, and contained everywhere brown pigment. Only at the base of the umbilicus, where the papillae were not markedly formed, was the pigment absent. Everywhere in the corium and in the subcutis were numerous mast cells. Hair and sebaceous glands were nowhere to be found. The deeper layers of the skin contained normally formed sweat-glands. The tumor consisted chiefly of a connectivetissue stroma and of cavities varying in size and form. The stroma, which in amount predominated over the alveolar tissue, was composed of broad, thick, dense connective tissue, which contained a few cell-elements with spindle-shaped nuclei. Only around the spaces there was present a connective tissue which was very delicate and whose fibers formed a network partly as fine bundles. The numerous nuclei were oval and frequently almost round. Immediately around the alveoli the connective-tissue threads formed a thick layer, really a membrana propria. The cavities were lined with cylindric cells placed at right angles to the basement-membrane. Their height was not always in proportion to the size of the cavity, but seemed to depend on the pressure of the gland contents. In a few places the tubules were filled with epithelium. The gland tubules were usually cut either obliquely or longitudinally. The gland lumina near the periphery of the tumor in width resembled normal sweat-glands. On the other hand, those in the middle of the tumor were markedly dilated and round; in the latter the tissue was frequently infiltrated with cells. The majority of the glands were filled with a secretion com26


386 THE UMBILICUS AND ITS DISEASES.

posed of a most delicate, rather granular network of threads mixed with epithelial cells. The entire tumor was permeated by a thick network of capillaries which surrounded the individual gland tubules. In many places in the connective-tissue stroma in the neighborhood of the blood-vessels were remnants of old and fresh blood.

In the preparations taken from the lateral portion of the tumor accumulations of round cells and blood-vessels were seen. The cystic dilatation of the canals had evidently been produced by pressure from within. The cavities were lined with endothelium, and the walls of these new cavities had projections into them. These cavities were due to the confluence of the neighboring small cavities. The origin of these in some places could be followed. At several points between the bloodspaces were dilated tubules lined with cylindric epithelium, usually filled with secretion, and surrounded by the characteristic connective tissue which sometimes reached as far as the endothelium of the blood-spaces. A few of the gland-like cavities also contained blood. At no point, however, was this adherent.

After these findings we must ask: Are we dealing here with an individual tumor or is there a combination of two tumors? Further, under what category does this tumor formation belong? Wullstein held it to be a combination of cystadenoma of the sweat-glands with cavernous angioma.

On p. 250 he says that what makes him think there is a combination of two tumors is the fact that there is a different lining to the large spaces, the one being lined with endothelium and the other with cylindric epithelium. No less typical is the relation of the surrounding connective tissue to the spaces. The differences even with the low power are easily recognized, through the various microchemical reactions in color with methylene-blue. The above already described delicate bluish connective tissue is independent of the sweat-glands and their tributaries in the specimen, and is present only in the vicinity of the tubules lined with cylindric epithelium, whereas the spaces lined with endothelium are always surrounded by a thick, fibrillated tissue which stains intensely red. He thinks that the large cavernous spaces in the first place are due to circulatory disturbances.

On p. 251 he says we must look upon the sweat-glands as the point of origin for the epithelium of the new-growth, on account of the position of the tumor beneath the skin, the presence of cylindric epithelium, and the absence of squamous epithelial nests. Its origin from the epidermis or from the hair-follicles or the sebaceous glands is excluded. On the other hand, we must ask whether it may not be due to some embryologic deposit. Three things have to be thought of: the umbilical canal, the urachus, and the omphalomesenteric duct. Have we in this mixed tumor a purely accidental combination of an adenomatous cyst of the sweat-glands and a cavernous angioma? or do the two varieties bear a causal relation one to the other? In conclusion, he says, the old and fresh hemorrhages in various portions of the tumor have followed as a result of hyperemia — perhaps the menstrual hyperemia. [Wullstein's tumor also occurred in a woman. He speaks of its characteristic connective tissue separating the glands lined with cylindric epithelium from the surrounding stroma. Further, in his last paragraph he speaks of the hemorrhage through the tumor being due to hyperemia, possibly menstrual in origin. We believe that here he has the clue and that, in all probability, the glands in this case were also uterine glands. Although the description of the histologic appearances in this case is in places somewhat involved, we have in our translation held closely


ADENOMYOMA OF THE UMBILICUS. 387

to the text in order that the points favoring the uterine origin of the glands might not be unduly accentuated. I wrote Professor Orth, of Berlin, and he in turn referred me to Dr. Wullstein, who at the time this case was published (1893) was an assistant of Professor Orth and occupied the room next to mine in the Gcittingen Laboratory. Dr. Wullstein kindly sent me the reprint of his article, but I was unable to get the specimen, and consequently cannot speak with absolute certainty.— T. S. C]

N. Giannettasio, in an article,* gives a resume of the literature on tumors of the umbilicus, and reports a case in a multipara aged forty-four. A year and a half before she came under his observation the patient noticed a small tumor the size of a walnut at the umbilicus. This was solid, immobile beneath the skin, and occasionedno discomfort. It occupied the lower andleft side of the umbilical depression. It was removed, and the patient was perfectly well twenty-five months later. He gives a very good plate, but the text is not satisfactory. The nodule, however, he says, contained "cytogenous" connective tissue. The plate shows normal skin, dilated blood-vessels, and gland-spaces lined with apparently cuboid epithelium, and surrounded by a stroma, the picture somewhat suggesting uterine glands.

Probably Uterine Glands in a Small Umbilical Tumor, f — In the beginning of his article von Noorden states that he is going to demonstrate a tumor which, from its characteristics and anatomic picture, leaves no doubt that it originated from the sweat-glands, and that, so far as he knew, no similar case was on record. On October 1, 1898, a thirty-eight-year-old multipara told him that for two months she had had a slight unevenness in the middle of the umbilicus. Eight days previously a physician had observed a pea-sized enlargement in the floor of the umbilicus. Clinically it suggested a nevus, and on account of the dark pigmentation von Noorden thought of melanosarcoma. On October 14, 1898, the tumor was larger than a pea, semicircular, and not sharply defined from the surrounding umbilical tissue. In its center it had a small, wart-like elevation. There were no inflammatory changes in the vicinity. The skin over the tumor was somewhat uneven, grayish in color, and here and there more deeply pigmented than the floor of the umbilicus. No pulsation was noted, no variation on pressure. The umbilicus was removed. Two and a half years later the patient was perfectly well.

The umbilicus on section showed a drawing in of the skin, and in the depth there was a wart-like projection. The tissue of the umbilicus itself was very hard. On section a pea-sized, light brownish, pigmented area was observed, which was not sharply defined from the surrounding tissue.

Microscopic Examination. — The nodule was made up of a loose connective tissue with numerous large cells. It contained a large number of capillaries. Within this connective tissue were slit-shaped cavities lined with cylindric epithelium which had become loosened irregularly from the wall. Some of these cavities had become dilated into irregular cystic spaces, which here and there showed clearly a lining of cylindric epithelium, while in other places they had completely lost it. The contents of these cavities had dropped out in some places; in others it consisted of cylindric epithelium, and in numerous cases of an irregular, structureless network. Further sections were made, and the squamous epithelial layer over

  • Giannettasio, N. : Sur les tumeurs de l'ombilic. Arch. gen. de nied., 1900, n. ser., iii, 52.

t von Noorden, W. : Ein Schweissdrusenadenom mit Sitz im Nabel und ein Beitrag zu den Nabelgeschwtilsten. Deutsche Zeitschr. f. Chir., 1901, lix, 215.


388 THE UMBILICUS AND ITS DISEASES.

the entire nodule was found to be intact. Over the most prominent part it was three times as thick as at the periphery. Where the cells were most abundant, the deepest layers showed pigmentation. At one point (Fig. 178) "the sweatglands ' ' could be traced almost to the surface, being covered only with a few layers of cells.

The stroma consisted of three definite kinds of tissue : normal, dense fibrous, and mucoid-like tissue. The chief interest lay in the sweat-glands ; roots of hairs were nowhere to be found, and sebaceous glands were reduced to a minimum. The search for muscle-fibers in the reticulate.d tissue was fruitless. No elastic fibers were found.

In general it ma}^ be said the sweat-glands were normal in the subcutaneous layer and were arranged in groups. Then in one section one would find two large openings and three or four glands, and in another section groups of from two to four glands. Some were cut in such a manner that 9 to 15 round lumina were in a









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Fig. 178. — A Small Umbilical Tumor Containing Numerous Glands. (After von Noorden.) This is a low-power picture of the mass. The growth is covered with squamous epithelium. Scattered throughout the stroma are quantities of glands. In form they bear a closer resemblance to uterine glands than to sweat-glands. At one point the glands almost reach the surface. (For a higher magnification see Fig. 179.)

line or in the form of a hook. The groups lay, as a rule, very close to one another. The normal sweat-glands lay partly in the fibrous connective tissue, others — and this is to be noted — were separated by a rather broad layer of cells from the normal corium. The nuclei of this zone were pale and less abundant than in the remaining corium. This zone suggested the above-mentioned mucoid tissue, in which in part the altered glands lay. This tissue appeared always to penetrate between the normal gland grouping, and had separated the glands from one another. The gland epithelium was not changed. In addition to this slightly normal and slightly changed skein-like gland there were in the corium a number of cavities and tubules. These extended from near the surface of the papillary masses to the vicinity of the subcutaneous fat. The cavities and the tubules are to be seen in Figs. 178 and 179. [We do not clearly understand what von Noorden means by corium. It seems, however, that he uses the term instead of stroma. His general description is somewhat hazy throughout. — T. S. C]


ADENOMYOMA OF THE UMBILICUS.


389


On p. 222 he gives a resume of his description: The tumor is made up of many roundish and often dilated, cyst-like portions which lie deeply seated in the corium. In intimate relation to these, or independent of them, are tubular channels with numerous corkscrew-like windings. These extend toward the epidermis. The cystic and also the tubular pictures are surrounded by dense and loose connective tissue which separates them from the surrounding connective tissue and are without any definite capsule. In the above-described coil we can with certainty recognize the sweat-glands.

On p. 229 he reports one of Mintz's cases and says that possibly the new-growth had developed from the glandular portion of the skin; for example, from the sweatglands. He says: "I will also not assume this, but will say that portions of my tumor in respect to form, grouping, contents, and relation of the cells, both in the description and in the picture, produce a very similar appearance to the case reported by Mintz, and had it not been possible to establish a relation to the sweat-glands I should in all probability have followed the views of Mintz. Mintz found smooth muscle-fibers in the connective tissue at several points. The explanation as to the origin is difficult. " In conclusion, von Noorden says: "From the above findings a true benign adenoma springing from the sweat-glands can be diagnosed."

[As will be noted from the history, the patient was a woman thirty-eight years of age. There was no evidence of inflammation. Histologic examination in some places showed groups of glands lying in a stroma differing from the ordinary surrounding stroma. These groups of glands were lined with one layer of cylindric epithelium, and the cavities of some of the dilated

spaces contained cells that had taken up blood-pigment. Yon Noorden draws attention to the fact that his case bore a marked resemblance in many ways to Mintz's case. There remains little doubt in my mind that the glands resemble those found in the body of the uterus, and the thickened, dense stroma around them bears a marked resemblance, even with the very low power, to the stroma of the uterine mucosa. The picture, at any rate, is much more suggestive of a glandular growth of uterine origin than of one coming from the sweat-glands. I endeavored, through Professor Doderlein, of Munich, to locate Dr. von Noorden, and, if possible, secure a section of this growth, but have not been successful. — T. S. C]


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Glands in a Small Umbilical Tumor. von Noorden.) . The glands in the lower half of the picture bear quite a resemblance to uterine glands. Those in the center of the field remind one of the pictures seen in the depths of uterine glands, where there is some reduplication of the folds. The gland in the left part of the field is markedly dilated and contains much detritus.


390 THE UMBILICL T S AND ITS DISEASES.

It is rather difficult to classify this tumor reported by Villar, but as it presents a few clinical and histologic points suggestive of the group under consideration, I mention it here, although it is not considered in the digest.*

L. L.. aged forty-six. entered the service of Professor Guyon September 17, 1886. In the month of December, 1885, nothing abnormal was noticed in the umbilical region, but shortly afterward her corsets produced pain in this region and she discovered a small tumor the size of a pin-head, reddish in color, in the umbilical depression. This tumor increased very slowly, and in May, 1886, she went to the hospital for examination. She continued under treatment, and in the month of August entered the hospital. At that time at the umbilical depression was a tumor the size of a bird's egg. It was conic. Its base was continuous with the cicatrix, and was somewhat constricted by the depression. It had a very narrow, but relatively large pedicle. It was in reality sessile, firm in consistence, but elastic and reddish in color. At the top was a blackish point, 2 mm. in diameter. The tumor itself was not ulcerated and did not discharge any liquid. Two or three days after she entered the hospital the blackish point ruptured and there was an escape of tarry blood. The patient experienced no pain and there was no glandular enlargement.

Histologic Examination by Clado. — The tumor is situated in the center of the umbilicus and has developed in the depth of the cicatrix. It is covered with skin. In consistence it is a little less firm than a fibroma. On section one finds a capsule which surrounds the central mass. The tumor is whitish-gray, with numerous dark spots not any larger than the head of a pin scattered throughout it. Microscopic examination shows that the tumor is formed of sarcomatous tissue, the cells being fusiform in shape.

Some of the spaces are round, others oval, and have anastomosed with one another. Some of the canals are lined with pavement epithelium. Between the cystic spaces one finds stroma containing a small number of vessels. The skin which composes the outer covering of the tumor is exceedingly thin, but presents the characteristic appearance. There has been extravasation of blood at the center of the tumor.

[This woman, as above noted, was fortj^-six years of age. The history does not convince one absolutely that this was a sarcoma. It might very well have been a fibroma. It resembles in a few particulars those tumors of the umbilicus that contain uterine glands or glands somewhat resembling them. — T. S. C]

Further Cases of Adenomyoma of the Umbilicus. These four cases have come to my knowledge since this chapter was prepared.

They bear a striking resemblance to those already discussed in the preceding

pages :

T w o U in b i 1 i c a 1 T u in o r s of Probable Uterine O r i g i n . f " In the surgical service of Drs. Munro and Bottomley, at the Carney Hospital,

there recently occurred within a few weeks of each other two examples of umbilical

tumor, the striking similarity and unusual histologic structure of which warrant

their publication.

  • Villar: Tumours de l'ombilie. These de Paris, 1886, obs. 68.

t ( roddard, Samuel W.\ Surg., Gyn. and Obst,, August, 1909, 249-252.


ADENOMYOMA OF THE UMBILICUS. 391

"Because of the comparative rarity of these cases the clinical histories are set forth in considerable detail :

"Case 1. — Miss S., a housekeeper, forty-four years of age, and born in New Brunswick, entered the Carney Hospital May 22, 1907. Her family and past history have no bearing on her condition at that time. A year previously, during a catamenial period, she noted some redness and tenderness about the umbilicus; two months later, at a similar time, a small tumor appeared in the abdominal wall close to the umbilicus. This tumor increased in size but slightly, and most of the increase came in the two weeks just preceding her admission to the hospital. The tenderness and pain, which at first were evident only during the menstrual periods, had been constant for some months, though most marked just before, during, and for a week after menstruation. Her menstrual history w r as not otherwise remarkable. An abdominal bandage, her only treatment, had given her some relief. There had been some little loss of weight and strength. For two months the tenderness had kept her from her usual work. No symptoms referable either to the gastro-intestinal or to the urinary tract had been noted.

"About and including the umbilicus was a rather deep-seated, spheric, slightly tender, fixed mass, of rather firm consistence, and about 2 cm. in diameter. In the navel itself was a thin, yellowish crust; a sinus could not be demonstrated; the skin over the tumor was not red. Examination of the abdomen was otherwise negative. Examination per vaginam showed only vaginismus and a moderately retroverted uterus.

"On May 23d Dr. Munro excised the growth (including the navel) with a portion of the adjacent peritoneum and sheath of the rectus muscle. The former was not involved in the growth; to the latter the growth was adherent. The convalescence was without note, and the patient was still free from recurrence one year after operation.

"Case 2. — Mrs. D., a housewife, entered the Carney Hospital June 23, 1907. She was born in Ireland forty-two years before that time, and came of healthy stock. Her menstrual history previous to her marriage was entirely normal in every way. Married seventeen years, she had borne four children. Following her first confinement she had had a ' milk leg. '

"For six years previous to entering the hospital a slight bloody discharge from the navel without pain or tenderness had come with each menstruation. The discharge came only at that time. Independent of the umbilical disorder she had had in the past three years attacks of sharp pain beneath the right costal border, accompanied by vomiting, chills, and jaundice.

"The patient was rather obese, and showed distinct tenderness beneath the right costal border. At the umbilicus was a small, irregularly shaped papillomatous tumor, 2 cm. in diameter, with three distinct projections covered with normal appearing skin. At the top of the largest projection was a pin-hole opening capped with dried blood. The tumor was soft, freely movable, not tender, and apparently superficial.

"On June 24th the umbilicus with the tumor was excised by Dr. Bottomley. The tumor was confined to the skin and fat outside the aponeurosis. The peritoneal cavity was opened, and the gall-bladder and stomach regions were explored; these were found normal. Convalescence was uneventful except for the development of malaria on the ninth day, which promptly yielded to treatment. The


392 THE UMBILICUS AND ITS DISEASES.

patient was discharged, relieved, on July 11th, and when heard from, one and a half 3 r ears later, there had been no recurrence.

"For the microscopic study of these tumors, in the laboratory of Dr. Henry A. Christian at the Harvard Medical School, a large number of sections were taken from different planes and four different methods of staining were used for each section.

"So closely do the tumors resemble each other microscopically that no evident difference between them can be determined. The arrangement and construction, both in general and particular, are nearly identical. For descriptive purposes a median longitudinal section of Case 2 will be used. To the naked eye it presents an irregularly convex surface covered with true skin. Underlying this at each extremity are what appear to be sweat-glands, and in another part, chiefly in the center, are numerous vacuolated structures varying in size from a pin-point to a pin-head. The intervening structure cannot be definitely determined. Microscopically, the tumor is seen to be covered with normal epidermis, but varying in thickness. Below this, at either end, are numerous sweat-glands, thickly grouped, and around these is an abundance of fibrous connective tissue. The vacuolated or glandular structures found throughout the tumor vary in size, and for the most part are of rounded contour, while some are elongated. Some, especially the larger ones, are discrete, while others are aggregated into small groups. Some are immediately surrounded by fibrous tissue, while others are embedded in cellular tissue. There are none which appear to have any connection with the epidermis. All the gland-spaces are lined with epithelium. They are either devoid of contents, or contain a granular, structureless material in which are often found groups of red bloodcells. The epithelium varies in the different glands and even in the same gland, from the low, flattened variety to the tall, columnar cells with all the intermediate forms. The tall, columnar variety is for the most part closely compacted, with long, narrow nuclei and with no visible cell membrane. Most of them have a distinct top plate, and many show cilia of considerable length and uniformity, while others have only a suggestion of striae. The cilia in some places are from onefourth to one-third the length of their cells, and in others their extremities end in a globular, deeply staining tip. At irregular intervals among the nuclei of the columnar cells are larger rounded and more faintly stained nuclei. In some places the epithelium is distinctly cuboid, the nuclei clear and rounded, and the whole cell clearly defined. There is a larger group of glands which presents the flattened epithelium. The epithelium lining the glands, whether flattened, cuboid, or columnar, is for the most part in single layers. In some places the glandular epithelium is immediately supported by fibrous connective tissue, but in others the underlying structures are decidedly cellular. The cellular tissue is more compact the nearer the glandular tissue is approached, i. e., the most cellular tissue is found in close connection with the gland-spaces. The nuclei are rounded or elongated and deeply stained, the protoplasm and cell membrane not being distinct. In the immediate neighborhood of some of the gland-spaces are large hemorrhagic areas in which large quantities of red blood-cells are scattered freely and intermingled with the cellular structures. These areas seem to have no direct relation to blood-vessels, which are not superabundant or enlarged. The fibrous connective tissue shows nothing of interest throughout the section. There is an abundance of smooth muscle which is closely interwoven with the connective tissue."


ADENOMYOMA OF THE UMBILICUS. 393

The microphotographs accompanying Goddard's article bring out clearly the structure and arrangement of the tumors, and emphasize the points mentioned above.

Adenomyoma of the Umbilicus; also a S m all Adenomyoma near the Anterior Iliac Spine.* — Case 3. — "A woman, aged thirty-seven, came to me on September 2, 1908, for advice about a small tumor of the umbilicus which she had noticed during the last few months. The lump was about the size of a filbert, and lay in the lower part of the navel. It was irregular in outline, but smooth, and was of a bluish-purple color, suggesting a melanotic sarcoma. There were no abdominal symptoms or signs and no secondary deposits in the inguinal glands or elsewhere. A few days later I removed the whole navel and adjacent skin widely between two elliptic incisions, opening the abdomen on either side and taking away the intervening peritoneum. There were no traces of growth within the peritoneal cavity. The wound was stitched up in layers and healed absolutely by first intention. The specimen was given to Air. Lawrence, the curator of our museum, for examination. Sections showed to the naked eye a hard, fibrous structure, the superficial parts of which, under the epithelial covering of the navel, were pigmented. In the deeper parts of this fibrous tissue were many islands of tubular glands lined with columnar epithelium and filled with epithelial debris. Some were cut obliquely and showed a looser areolar investing layer outside the membrana propria. The latter was not penetrated by the cells, so that one sign of the benign character of the tumor was present. Nor were there any other signs of the spread of the growth beyond the limits of the tubules. L therefore, put it down as an adenoma derived from remnants of the vitelline duct, of which I had read but never seen.

"I saw no more of this lady until January, 1913, when she consulted me about a little nodule seated in the subcutaneous fat, about two inches internal to the left anterior iliac spine. It felt about the size of a pea, and was hard. On gently pinching the skin the latter puckered over the nodule. There were no enlarged inguinal glands or other signs of infiltration. This knot was removed shortly after by Mr. F. Hinds, of Worthing, and was sent to me. Mr. Lawrence kindly prepared several microscopic sections of it. They showed precisely the same structure as the first nodule, except that the fibrous tissue, which made up the bulk of the mass, was more dense and fewer connective-tissue corpuscles were scattered through it.

"The reappearance of this small knot, repeating the structure of the first nodule at the umbilicus, suggests, of course, strongly that the first was malignant and has recurred in the lymphatics of the subcutaneous tissue of the abdominal wall. Then the question arises, Was the original lump in the umbilicus a primary growth in some of the glandular remnants of the umbilicus enumerated above, or could it be a nodule secondary to some visceral carcinoma within the abdomen? This latter view is one adopted by Mr. Shattock, to whom I sent sections of both the first nodule removed and that obtained four and a half years later, and who was kind enough to write to me fully on the subject. It may be correct, but so far the lady has shown no evidence of visceral trouble — nearly five years after the appearance of the first nodule in the umbilicus. Time alone will show. In the meanwhile I am inclined to negative the visceral theory."

  • Barker, A. E. : Three Cases of Solid Tumours of the Umbilicus in Adults. The Lancet,

London, July 19, 1913, 128.


394 THE UMBILICUS AND ITS DISEASES.

In answer to a request from me, Dr. Barker very kindly sent the only section of the umbilical tumor which the curator of the museum still possessed.

Description of the slide sent me by Dr. Barker (His No. 10,945). — The section of the umbilical nodule has a normal covering of squamous epithelium. The underlying tissue shows no evidence of glandular tissue. Dr. Barker, however, in his description of the case, says that this tumor contained glands, and, furthermore, that the glands near the anterior-superior spine were similar in character to those found at the umbilicus. Dr. Barker was good enough to also send me several slides from


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Fig. ISO. — Adenomyoma in the Abdominal Wall near the Anterior Iliac Spine. This is a photomicrograph of a portion of the small nodule furnished me by Mr. Arthur E. Barker, London, England. Near the center of the field are two glands. Their epithelium has been slightly strengthened to bring them out more distinctly. The glands are lined with one layer of cylindric epithelium. Surrounding them is a zone of stroma cells. This zone is continuous with a large, irregular area of stroma just below and to the left of the glands. In the upper part of the field is another gland, which lies in direct contact with the tissue of the tumor. The greater part of the nodule consLsts of non-striped muscle and fibrous tissue. In the outlying portions of the field is adipose tissue. The growth is a typical adenomyoma, with glands similar to those of the uterine mucosa. Mr. Barker, in his description of the case, says that the umbilical nodule and the one here depicted were identical in character; consequently the umbilical growth was also an adenomyoma with glands and stroma identical with those of the endometrium of the uterus.

the growth near the anterior-superior spine. In one section I found not only myomatous tissue, but a triangular area of stroma with tubular glands at one end Tig. 180;. This area was sharply defined from the surrounding tissue. In another section was what appeared to be fibrous tissue, and possibly a little muscle. Here we had irregular, triangular areas of stroma, sometimes without any glands, sometimes with tubular glands identical with those of the uterine mucosa. At other points the glands lay in direct contact with the muscle. Surrounding the entire growth was adipose tissue. The picture in the main is analogous to that which we


ADENOMYOMA OF THE UMBILICUS. 395

have described as representing adenomyoma of the umbilicus. Mr. Barker's case is particularly interesting in that he had not only a tumor of this character at the umbilicus, but also a nodule near the anterior iliac spine.

A Small Umbilical Tumor Consisting i n P a r t o f Sweatglands and in Part Apparently of Uterine Glands.- — While in Atlanta, at the meeting of the Southern Surgical Association in December, 1913, Dr. Edward G. Jones, of Atlanta, told me that he had recently seen an umbilical tumor in which I might be interested. On December 22, 1913, he wrote: "I am sending under separate cover a section of the umbilical tumor. Unfortunately, I cannot give you any clinical data. The nodule was three-quarters of an inch in diameter, and gave the patient some discomfort at times." Later Dr. Jones discovered that, according to the patient's account, the tumor seemed to her to enlarge at the time of menstruation.

The specimen sent me by Dr. Jones is covered over with squamous epithelium which contains pigment in the deeper layers. The underlying tissue consists in a large measure of fibrous tissue. The capillaries scattered throughout it are in many places surrounded by round cells. Here and there throughout the fibrous tissue are groups of sweat-glands. These are separated from the fibrous tissue by a definite stroma.

At other points are large glands lined with cylindric epithelium. Some of these glands lie in direct contact with the fibrous tissue; others have a definite stroma, separating them from the connective tissue. This stroma stains more deeply than the connective tissue, and its nuclei are oval and stain deeply.

The tumor is evidently made up of two distinct varieties of glands: some corresponding to sweat-glands and others bearing a marked resemblance to those of the uterine mucosa. There is little doubt that part of this growth consists of uterine glands. The section was, unfortunately, too thick to supply a satisfactory photomicrograph.

PERSONAL OBSERVATION.

In 1900 Mrs. E. J. D., aged thirty-eight, was admitted to Dr. Howard A. Kelly's Sanitarium on account of a retroflexed uterus and a relaxed vaginal outlet. A small round nodule was at the same time detected at the umbilicus. The nodule was removed, the uterus brought up into position, and the perineum repaired. Her convalescence was prolonged on account of phlebitis in both legs.

This patient was the mother of four children. Her menses began at thirteen, were fairly regular, and lasted from three to five days. About two years before admission the patient first felt a little pain in the umbilical region. During the last year this had become very severe and the small umbilical growth had developed. There was no reddening at the umbilicus, and the general health had not been affected.

This small umbilical tumor was brought over to the gynecologic laboratory of the Johns Hopkins Hospital and carefully examined. For some unforeseen reason it was not indexed, and, consequently, when we were getting together all our umbilical material, was overlooked. It was accidentally discovered when class sections were being gone over a few days ago (March 3, 1915). Dr. Elizabeth Hurdon, who examined the specimen at the time, drew special attention to the fact that the


396


THE UMBILICUS AND ITS DISEASES.


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Fig. is). — A Small Umbilical Tumor Containing Glands Similar to those of the Body of the Uterus.

Gyn.-Path. No. X'.lll. This is a low-power photomicrograph of a section of the entire umbilical nodule. The skin covering is normal. Occupying the lower half of the field is a somewhat circular growth, denser in structure than the surrounding stroma. It consisted of fibrous tissue and non-striped muscle. Scattered throughout the tumor are glands. Some occur singly, others in groups. Some of the smaller glands are surrounded by a dark zone — a zone of characteristic stroma. Many of the glands are dilated and partially filled with blood. In the upper part of the field are aggregations of sweat-glands. (For the higher power picture see Figs. 182 and 183.)


ADENOMYOMA OF THE UMBILICUS.


397


glands in the growth were similar to those of the endometrium , and that some of them were surrounded by the characteristic stroma of the uterine mucosa.

Gvn.-Path. No. 39 14. The tumor averages 1.5 cm. in diameter.




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Fig. 182. — Adenomyoma of the Umbilicus. Gyn.-Path. No. 3914. This picture gives an enlargement of the adenomyoma seen in Fig. 181. The stroma of the growth consists of non-striped muscle and fibrous tissue. Occupying the center of the field are several glands. They were lined with one layer of cylindric epithelium, on which cilia were here and there demonstrable. The glands are separated from the muscle by a definite stroma. This, with a higher power, was found to be identical with that of the endometrium of the uterus. In the left upper corner of the picture is a markedly dilated gland. This and other dilated glands contained old blood and exfoliated epithelial cells, which had taken up blood-pigment and had become spheric. The entire picture of the umbilical tumor is analogous to that of an adenomyoma of the uterus.

Its outer surface is covered with normal-appearing skin. On section it presents a dense fibrous structure.

On histologic examination the skin surface is found intact and normal. The stroma of the growth consists of fibrous tissue with a moderate amount of nonstriped muscle distributed throughout it.


398


THE UMBILICUS AND ITS DISEASES.


Scattered here and there throughout the nodule are round or tortuous glands. Some of these occur in groups, others are single (Figs. 181 and 182). The glands are lined with one layer of low cylindric epithelium, which in a few places shows definite cilia. Some of the gland cavities are empty, others are dilated and filled with old blood, and in a few are exfoliated epithelial cells which have become spheric and have taken up the blood-pigment. Some of the glands lie in direct contact with the muscle or fibrous tissue; others are separated from the dense tissue by a



Fig. 183. — A Group of Sweat-glands in an Umbilical Tumor. Gyn.-Path. No. 3914. For their relation to the adenomyoma of the umbilicus see Fig. 181.


definite stroma, which is very cellular. The picture is that of a typical adenomyoma with glands identical with those of the uterine mucosa.

At one point is an aggregation of glands of a totally different type. These glands are small, round, and have a lining of two layers of low cuboid cells. They closely resemble sweat-glands (Fig. 183).

This is another definite example of an adenomyoma of the umbilicus. It will be remembered that in several of the recorded cases the sweat-glands were markedly increased in number.


ADENOMYOMA OF THE UMBILICUS. 399


LITERATURE CONSULTED IN THE PREPARATION OF UMBILICAL TUMORS CONTAINING UTERINE MUCOSA OR REMNANTS OF MULLER'S DUCT. Barker, Arthur E.: Three Cases of Solid Tumours of the Umbilicus in Adults. Lancet, London,

July 19, 1913, 128. Cullen, Thomas S.: Umbilical Tumors Containing LTterine Mucosa or Remnants of Midler's

Ducts. Surg., Gyn. and Obstet., May, 1912, 479. Ehrlich: Primares doppelseitiges Mammacarcinom und wahres Nabeladenom (Mintz). Aus

von Eiselsberg's Klinik. Arch, f . klin. Chir., 1909, lxxxix, 742. Giannettasio : Sur les tumeurs de l'ombilic. Arch. gen. de med., 1900, n. serie, iii, 52. Goddard, Samuel W.: Two Umbilical Tumors of Probable Uterine Origin. Surg., Gyn. and

Obstet., August, 1909, 249. Green: Trans. Path. Soc. London, 1899, 1, 243. Herzenberg: Ein Beitrag zum wahren Adenom des Nabels. Deutsche med. Wochenschr., 1909,

i, 889. Mintz, W.: Das wahre Adenom des Nabels. Deutsche Zeitschr. f. Chir., 1899, li, 545. von Noorden, W. : Ein Schweissdrtisenadenom mit Sitz im Nabel und ein Beitrag zu den Nabel geschwulsten. Deutsche Zeitschr. f. Chir., 1901, lix, 215. Villar, Francis: Tumeurs de l'ombilic. These de Paris, 1886. Wullstein, L.- Eine Geschwulst des Nabels. (Kombination von Cystadenom der Schweiss driisen mit cavernosem Angiom.) Arb. a. d. Path. Inst, in Gottingen, R. Virchow, zum 50.

Doctor-Jubilaum, 1893, 245.




Umbilicus (1916): 1 Umbilical Region Embryology | 2 Umbilical Region Anatomy | 3 Umbilical New-born Infections | 4 Umbilical Hemorrhage | 5 Umbilicus Granuloma | 6 Omphalomesenteric Duct Remnants | 7 Umbilicus Abnormalities | 8 Meckel's Diverticulum | 9 Intestinal Cysts | 10 Patent Omphalomesenteric Duct 1 | 11 Patent Omphalomesenteric Duct 2 | 12 Bowel Prolapsus at Patent Omphalomesenteric Duct | 13 Abdominal Wall Cysts by Omphalomesenteric Duct Remnants | 14 Omphalomesenteric Vessels Persistence | 15 Umbilical Inflammatory Changes | 16 Subumbilical Space Abscess | 17 Umbilicus Paget's Disease | 18 Umbilicus Infections | 19 Umbilicus Abnormalities 2 | 20 Umbilicus Fecal Fistula | 21 Umbilicus Round Worms | 22 Umbilicus Foreign Substance Escape | 23 Umbilical Tumors | 24 Umbilicus Adenomyoma | 25 Umbilicus Carcinoma | 26 Umbilicus Sarcoma | 27 Umbilical Hernia | 28 The Urachus | 29 Congenital Patent Urachus | 30 Urachus Remnants | 31 Urachal Remnants Producing Tumors | 32 Large Urachal Cysts | 33 Anterior Abdominal Wall Abscesses | 34 Urachal Cavities | 35 Umbilicus Acquired Urinary Fistula | 36 Urachal Concretions and Urinary Calculi | 37 Urachus Malignant Changes | 38 Urachus Bleeding into the Bladder | 39 Patent Urachus Tuberculosis | Figures

Reference

Cullen TS. Embryology, anatomy, and diseases of the umbilicus together with diseases of the urachus. (1916) W. B. Saunders Company, Philadelphia And London.

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