Book - Umbilicus (1916) 37
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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.
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Chapter XXXVII. Malignant Changes in the Urachus
Carcinoma of the urachus.
Report of cases. Sarcoma in the urachal region. An extraperitoneal abdominal tumor.
A large multilocular carcinomatous cyst of the urachus; secondary growths in the pelvis (personal observation). A rare umbilical cyst.
Carcinoma of the Urachus
I have been able to find three cases of carcinoma of the urachus recorded in the literature.
Sex. — All of the patients were men. Two of the patients had had congenital urinary fistula? at the umbilicus, and in each of these the discharge of urine had ceased after the use of escharotics. The third patient also evidently had a congenital fistula, as he gave a history of "moisture at the umbilicus" during childhood. This had ceased without treatment.
Age. — The patients were twenty-five, twenty-seven, and thirty-two respectively, indicating that, when carcinoma of the patent urachus develops, the malignant change occurs in early adult life.
Hoffmann and Fischer gave very careful and full histories of their cases. Hoffmann's patient, when twenty-seven years of age, noted a raised hardening between the umbilicus and symphysis. It was the size of a goose's egg, non-painful, and movable from side to side. It gradually extended toward the symphysis and right inguinal region.
Shortly after the tumor was noticed the patient experienced pain on urination. At times the urine was abundant, at times it came drop by drop. The man rapidly grew weaker and lost 25 pounds in four months. When Hoffmann saw him. the umbilicus presented a peculiar radiating appearance, while in the mid-line, just below the umbilicus, was a roundish, nodular tumor, 8 to 10 cm. long, adherent to the umbilicus and very painful. After the patient had urinated an area of tympany could be elicited between the tumor and the symphysis. On account of tenesmus, the patient urinated every hour. The urine contained pus and aggregations of epithelial cells.
The tumor became fluctuant, ruptured, and a large amount of purulent and bloody fluid escaped, but the growth did not diminish in size. From time to time onion-like balls escaped with the pus. These consisted of quantities of squamous epithelial cells that had become agglutinated. Precisely similar balls escaped in Fischer's case.
The urethra was normal.
The umbilical opening closed temporarily, but soon reopened, and in the late stages of the disease the inguinal glands were swollen.
As noted in the autopsy report, the cavity between the umbilicus and bladder had walls 1 cm. thick. Its inner surface had an irregular, ulcerated, and eaten-out appearance (Fig. 256). The bladder-wall had been involved by continuity, and also contained secondary nodules. The growth was a squamous-cell carcinoma.
Fischer's patient, when thirty-one years old, first noted a small, hard tumor the size of a pigeon's egg below the umbilicus. Seven or eight months later he had pain on micturition, and noticed a sediment in the urine. The nodule was incised on the supposition that it was fluctuant, and slimy, necrotic tissue escaped. The tumor soon grew out of the incision, bled a great deal, and finally left an ulcerated area, the walls of which were raised and hard, while the floor consisted of hard nodules. From the ulcerated area onion-like balls of epithelial cells escaped.
The inguinal glands on both sides became swollen. At autopsy the bladder mucosa showed a catarrhal swelling, but no involvement by the malignant growth. The prostate was normal. The growth was a carcinoma, evidently of the squamouscell type, as indicated by the onion-like balls.
Death in these cases may occur from gradual weakening as a result of the disease, or from a perforation of the growth posteriorly into the abdominal cavity, causing a peritonitis. The occurrence of three cases of carcinoma of the urachus is another point in favor of the early removal of the patent urachus.
In the future cancer of the urachus, when met with, will undoubtedly be operated on early. The growth can be given a relatively wide berth, and the block dissection should include the inguinal glands on both sides.
Cases of Carcinoma of the Urachus Developing Years After the Closure of a Congenital Patent Urachus.
Carcinoma Evidently D e v e 1 o p i n g F r o m Remains of the Urachus. — Fischer* saw this patient in consultation with Hanuschke in 1874. The man, thirty-two years of age, sought treatment on account of an ulcer of the umbilicus. During his childhood, when voiding, there was a moisture at the umbilicus. Later these symptoms disappeared and there was never any trouble with urination. Early in 1873 he casually noticed below the umbilicus a hard tumor the size of a pigeon's egg. This gave rise to no symptoms. It gradually grew, and seven or eight months later there were pain and a burning sensation on micturition and sediment in the urine. Toward the end of 1873 he consulted a physician. The difficulty in urination had increased, and the tumor had grown markedly. His general condition was not satisfactory. Hanuschke thought that the tumor was soft, and that he could make out fluctuation. Accordingly he made an incision, and purulent, slimy masses escaped — evidently pieces of necrotic tissue. The tumor mass grew out of the incision wound ; it very soon broke down, with a good deal of bleeding, and an ulcer resulted. When Fischer saw the patient, he was pale and weak, had difficulty in micturition, and suffered from strangury. The ulcer was situated 2 cm. below the umbilicus, and formed a deep crater, which was heart-shaped. Its walls were elevated, hard, and extended below the level of the skin about 4 cm. Its greatest breadth was 8 cm. Its greatest length, 7 cm. The floor was very irregular and covered with hard nodules. It reached a depth of 5 cm. below the skin surface. Surrounding the ulcer the tissue was hard. On pressure there escaped a thin, bloody, foul-smelling pus from the ulcer, and there were also portions of the tumor forced out as small balls suggesting onions. These were composed of quantities of flat epithelial cells.
Fischer: Die Eiterungen im subumbilicalen Raume. Volkmann's Sammlung klin. Vortrage, n. F. No. 89 (Chir. No. 24), Leipzig, 1894, 519.
The urine was acid, slightly cloudy, and had a purulent sediment. The inguinal glands on both sides were swollen.
Complete removal of the growth was impossible. The abdominal walls, however, were split in the mid-line as far as the symphysis, and beneath the muscle thick, pork-like tumor masses were found adherent. As much of the tumor as possible was removed, and the cautery was employed. The patient died fourteen days after the operation. Microscopic examination of the tumor mass showed it to be a carcinoma. At autopsy the inner surface of the bladder was found to show catarrhal swelling. It was intact. There was no abnormality in the prostate. The intestines were normal.
A Patent Urachus, Closure; Later Carcinoma of the Urachus.* — This case was also mentioned in the Deutsche Klinik, 1864, xvi, 116. The patient was a man, twenty-eight years of age, who had a urachal fistula at birth. This was healed with escharotics. Twenty-five years later a tumor developed between the umbilicus and the symphysis. This broke and discharged pus and later urine. The autopsy revealed a carcinoma of the mucosa of the urachus, which had perforated into the umbilicus and into the bladder.
A Patent Urachus Partly Closed by the Use of Escharotics; Later, Carcinoma of the Urachus. — Hoffmannf first reports the case of Hermann R., in which there was an enormous sac formation and accumulation of fluid outside of the abdomen. This Hoffmann attributed to a dilated urachus.
Hoffmann reports the case of Alexander Wanner, a postal employee, who was born in 1841 with an opening at the umbilicus through which urine escaped, while it also passed from the urethra. This condition lasted until his third year, when the opening closed after the use of escharotics. The patient had no further difficulty, and with the exception of several inflammations of the eye was perfectly well. About the middle of the year 1868 he noticed between the umbilicus and the symphysis, near the umbilicus, a raised hardening of the abdomen about the size of a goose's egg, which was not painful and could be pushed from side to side. This gradually grew and extended toward the symphysis, and spread toward the right inguinal region. Shortly after the appearance of the tumor the patient began to have pain on urination. The urine sometimes came in an abundant stream; at other times only in drops. As a result the patient had a continuous desire to urinate. The pains became severe and he grew weaker. He had lost weight — in the last four months, 25 pounds. On admission to the hospital, November 10, 1868, he weighed 99 pounds, was poorly nourished, anemic, and had a peculiar radiating formation of the umbilicus, in the folds of which no opening could be discovered. Immediately below the umbilicus was a tumor, 8 to 10 cm. long, situated in the middle line. It was roundish, nodular, very painful, and adherent to the umbilicus, but on both sides it was free. After urination, between the tumor and the symphysis was an area of tympany. On account of the tenesmus the patient urinated every hour, and the urine contained pus and aggregations of epithelial cells. The patient drank quantities of soda-water and local applications were made. His pain diminished, but the tumor continued to grow. The umbilicus became prominent, fluctuation was detected, and on December 1st the swelling broke and a large quantity of thick, purulent, bloody fluid escaped. The tumor, however, did not diminish in size, although the pain became less and less. In the fluid numerous onion-like balls were found. These consisted of large quantities of squamous epithelial cells which had become agglutinated.
Graf, Fritz: Urachusfisteln und ihre Behandlung. Inaug. Diss., Berlin, 1896. t Hoffmann: Zur pathologisch-anatomischen Veranderung des Harnstrangs. Arch. d. Heilkunde, 1870, xi, 373.
Examination of the urethra with a bougie yielded nothing abnormal. The prostate was not enlarged, the bladder-wall was thick and did not contract completely after the escape of urine. From September 4th urine and purulent fluid often escaped from the umbilicus, and the urine passed from the bladder from that time on was cloudy. The opening at the umbilicus gradually contracted, and for some time only purulent fluid escaped from it. The tumor became smaller, and toward the middle of January, 1869, the umbilicus closed completely.
Diarrhea developed and marked emaciation. At the end of January the opening at the umbilicus reappeared, and a purulent-like material escaped. The pain became more severe. The inguinal glands were swollen and the patient grew weaker. On January 31st he weighed 88 pounds. He died in the middle of May, 1869.
Only an incomplete autopsy could be obtained. The family physician who made it said there were appearances of peritonitis. The umbilicus had a peculiar, radiating, stellar appearance, and there was an opening 3 mm. in diameter. Through this there was a passage going downward and backward into a canal which gradually widened. The cavity had walls 1 cm. thick. It extended from the umbilicus to the top of the bladder. It was 10 cm. in length, and in its middle portion was 2.5 cm. broad. The entire inner surface presented an ulcerated, irregular, much eaten-out, reddish appearance (Fig. 256).
At its lower part this cavity communicated with the bladder by an opening 3.3 cm. broad, and the posterior wall of the bladder was invaded by this ulcerated growth over an area 4 cm. in diameter. The bladder-walls, where invaded, were 1.8 cm. thick, while the unchanged portions were 0.8 cm. thick. At the point where the cavity communicated with the bladder posteriorly was a perforation, the exact size of which could not be determined on account of the tearing of the specimen. The bladder mucosa, on the whole, looked normal, but at one point in the anterior wall was a round nodule, 1 cm. in diameter; in the posterior wall were several smaller ones.
Microscopic examination showed that the growth of the urachus was a squamouscell carcinoma, and that the secondary nodules were also carcinomatous.
Hoffmann says that this patient was born with a patent urachus. The opening at the umbilicus had closed after the use of escharotics in the third year. In the twenty-seventh year a carcinoma developed in the urachus and extended to the bladder. The perforation caused by the cancer led to a local peritonitis.
A Urachal Cyst and Cancer of the Bladder Occurring Independently. — ■ Rotter's case may well be considered here. The urachus was the seat of a cyst and the bladder showed a carcinoma. The one was absolutely independent of the other.
Fig. 256. — Carcinoma of the Patent Ubachus. (After C. E. E. Hoffmann.) A is a partially diagrammatic picture: ", The anterior abdominal wall; b, the opening of the urachus at the umbilibe urachus, which is occupied by a carcinoma; at d the growth has broken through into the abdominal cavity; < , the bladder. At points /, /, /, /, on the bladder mucosa are small secondary carcinomatous masses. B represents the appearance of the umbilicus with the opening of the urachal fistula in its center.
Rotter's patient was a forty-three-year-old man, who, for nine months, had had bleeding from the bladder. Cystoscopic examination showed a tumor in the upper portion of the bladder. This did not grow rapidly. Above the symphysis, and reaching to the umbilicus, was another tumor, which on aspiration yielded a fluid containing cholesterin. This tumor was diagnosed as a urachal cyst. At operation the upper tumor was found lying between the peritoneum and the abdominal muscles. In its upper portion it was free, but over the lower half it was so intimately blended with the peritoneum that it was necessary to remove a portion of the peritoneum with the tumor. The urachal tumor pressed so into the bladder muscle that it was also necessary to open this viscus.
The cancer of the bladder was removed, and a defect 7 by 8 cm. in the bladder closed by layers. This patient was shown by Rotter at the Berlin Surgical Society. Microscopic examination demonstrated carcinoma of the bladder. This had perforated at the point where the cyst was found. The cyst contained many polymorphous epithelial cells. There was no doubt that it was a urachal cyst.
Possibly an Adenocarcinoma of the Urachus. — I am at a loss where to place this case of Koslowski's.f The situation of the tumor suggests a urachal growth. Furthermore, the variation in the size of the glands might very readily correspond to the cyst-like spaces we have noted where isolated segments of the urachus have persisted. The invasion of the rectus sheath and of the rectus muscle naturally points toward malignancy. We shall accordingly leave this case among those of carcinoma of the urachus. Whether it really belongs here or not is problematic.
The patient was a man, fifty-five years of age, who five weeks before had noticed in the mid-line, between the symphysis and the umbilicus, a small, painful tumor which grew to the size of a walnut. This man was markedly emaciated, looked to be seventy years of age, had frequent diarrhea, and was bent over from guarding the abdominal muscles. Between the umbilicus and symphysis, near the mid-line, was a tumor which suggested a patella. The overlying skin was free. The tumor was slightly movable and very painful. It felt very tense, and gradually merged into the surrounding tissue. Passing from the tumor toward the umbilicus was a cord the size of a goose-quill. Koslowski thought the tumor was a malignant epithelial growth developing from remains of the urachus.
Operation. — A median incision showed that the linea alba and sheath of the rectus had been penetrated by the tumor. An elliptic incision encircled the umbilicus and the tumor. Removed with the tumor were portions of the sheath of the recti and some of the rectus muscle, the transversalis fascia, and peritoneum. After the abdomen was opened, the tumor was drawn up and brought into view fibrous cords passing to the umbilicus. The upper cord was the size of a goose-quill, firm, and infiltrated. The lower cord was less firm and contained veins; these passed into the vesico-umbilical ligament. The peritoneum covering the posterior surface of the tumor showed evidence of scar and of ulceration. The patient made a good recovery. The tumor in form resembled a patella. The peritoneum was firmly attached to it. The surrounding muscle was penetrated by the tumor. Microscopic examination showed that it was made up of glands of various sizes. They varied from the size of urinary tubules to those large enough to be noted with the naked eye. The diagnosis was fibro-adenoma submalignum. The glands resembled intestinal glands.
[It is difficult to establish the exact character of this tumor. — T. S. C]
Rotter: Blasencarcinom combinirt mit Urachuscyste. Centralbl. f. Chir., 1897, xxiv, 604. t Koslowski, B. S.: Ein Fall von wahrem Nabeladenom. Deutsche Zeitschr. f. Chir.. 1903, lxix. 469.
Frank, in 1893, recorded a very interesting case of sarcoma probably developing in the sheath of the urachus in a young lad. Unfortunately, the subsequent history of the case is lacking, but the histologic picture of the growth, the invasion of the muscles of the abdominal wall, and the secondary nodules in the omentum leave no doubt as to its malignancy.
Alban Doran reports a case of sarcoma developing in the wall of a cyst of the urachus. This is so interesting that I shall also record it in detail.
Sarcoma Probably Developing in the Sheath of the Urachus. — Frank* gives a good resume of the literature and reports the case of a boy eleven years of age. For several weeks he had had loss of appetite and was losing weight. About fourteen days before the boy came under observation the father noticed a swelling in the umbilical region, and from a small opening at the umbilicus a little pus could be pressed. There was no urinary difficulty and no discomfort on defecation. The urine, however, had recently became cloudy and stringy. The child's mother had died of pulmonary disease, otherwise the family history was good.
On examination the boy was found to be strong and well nourished. In the umbilical region was a hard, circumscribed thickening, only slightly painful on pressure, reaching about a fingerbreadth above the umbilicus. Here it could be traced three fingerbreadths to the right and to the left of the linea alba. Below it extended almost to the symphysis. The skin over the tumor was only slightly movable. A sound introduced into the sinus passed from 4 to 6 cm. downward. With a sharp curette friable, sanguineopurulent masses were removed. These on examination were found to consist of pus-cells, granulation tissue, and debris.
Operation. — An elliptic incision was made, commencing 3 cm. above the umbilicus. The recti muscles at the umbilicus were found to be infiltrated by the growth. The incision was then carried through healthy muscle to the peritoneum. Loops of small bowel were adherent to the peritoneal surfaces of the tumor, and nodules were found scattered throughout the omentum. The tumor was gradually turned out ward and was removed without much difficulty. Its lower end was intimately adherent to the bladder, and the outer walls of this viscus were removed and the small opening in it was closed. The omentum was removed on account of the tumor nodules. The abdomen was closed with difficulty. The patient's recovery was slow.
The tumor, on section, was found to have invaded the recti in all directions. Its chief extension was along the course of the urachus as far as the bladder. The tumor itself, with the surrounding parts, was as large as a man's fist, and was nodular and uneven.
Frank, Theodor: Zur Casuistik der Urachustumoren. Inaug. Diss., Wurzburg, 1893.
On microscopic examination the sarcomatous character of the tumor was evident. In the center of the tumor the intercellular substance was most marked, but toward the periphery it consisted almost entirely of spindle-cells with little connective tissue. The growth of the spindle-cells into the recti and into the bladder was especially evident. The entire picture indicated that the tumor had developed in the connective-tissue layers of the urachus and that it had then spread out in all directions.
The case is perfectly clear, but there is no after-history beyond two months, and no description of the omental nodules.
AUniqueSpecimenofCystic Sarcomaof the Urachus.* — Alban Doran says: "Mr. F. S. Eve has presented to the Museum of the Royal College of Surgeons of England a unique specimen of cystic sarcoma of the urachus, and has kindly supplied me with the following notes :
"A man, aged thirty-eight years, was admitted into the London Hospital with a swelling in the hypogastrium noticed for several weeks and associated with pain after micturition. A cystic tumor filled the lower part of the abdomen, especially to the right, where it extended toward the loin. It did not dip into the pelvis. On puncture, dark blood came away; a few days later a rigor occurred, with vomiting and a rise of temperature to 104° F. Mr. Eve then operated, exposing a large cystic tumor; the parietal peritoneum was reflected over its anterior and superior surfaces. Five pints of dark, bloody material were removed. The cyst adhered to the omentum, which bore engorged veins, and to an inch and a half of small intestine which was infiltrated where adherent. The adherent portion of the wall of the gut was excised, and the wound closed with sutures. The lower part of the cyst was intimately connected with the bladder, the serous coat of which organ was reflected onto its surface. This peritoneal covering was divided, and the cyst carefully dissected away from the bladder. During the process the bladder was opened, for the vesical wall at this point was so thin that the cavities of the cyst and the bladder were only separated by the vesical mucous membrane covered by a few muscular fibers. The opening was sutured, but not without great difficulty, owing to the thinness of the walls at this point. The sutures were further protected by gauze packing. A gauze drain was passed into the pelvis, and a catheter retained for a while in the bladder. Neither flatus nor feces could be made to pass after the operation, and the patient died on the fourth day. There was no general peritonitis, but the pelvic peritoneum had become inflamed at the point where the gauze had been applied.'
"Mr. Eve examined the specimen and found that it was a large allantoic cyst separated from the posterior superior surface of the bladder by nothing except a very much thinned mucous membrane. Their cavities, however, did not. communicate. The inner wall of the cyst was lined at certain points with very vascular polypoid masses, which proved to be, on microscopic examination, sarcomatous. The most unusual feature of this cyst was its malignancy, but its peritoneal relations were of greater importance in respect to the subject of this communication."
An Extra-peritoneal Abdominal Tumor
The following interesting case, the specimen from which was exhibited by Dr. Aveling, may be considered here, although from the description one could not say that the growth was a sarcoma. It may serve, however, to form the nucleus around which similar cases may be collected.
Doran, Alban H. G.: The Lancet, 1909, i, 1304.
Dr. Aveling* exhibited before the British Gynecological Society a subperitoneal tumor which had grown in the anterior abdominal wall and reached from two inches above the umbilicus to the pubes. It was removed after death, the patient having succumbed after an exploratory operation. Sir Spencer Wells, who saw the tumor, said he had seen only two similar cases, and he classified the tumor, according to Virchow, as a fibroma molluscum cysticum abdominale. The specimen was referred to Mr. Bland-Sutton and Dr. Aveling for further examination.
The tumor was ovoid in shape, and measured 10 inches in length, 7 inches in width, and weighed 4% pounds. It was surrounded by a distinct, thick, fibrous capsule. On section the tissue was of a dirty white color, and the cut surface looked like a sponge. The loculi were filled with gelatinous tissue, which readily broke down on scraping the cavities with the handle of a scalpel. Inside the growth six or seven hard nodules, of the size of walnuts, could be felt. These, when dissected out and divided, looked like small leiomyomata, such as occasionally exist in the uterus. They presented the same whorled arrangement of the fibers, and corresponded with them histologically. On microscopic examination of the tumor the outer portion was found to consist of non-striped muscle-fibers, some of large size. Internal to this the cells assumed more the shape and characters of those seen in spindle-cell sarcomata, while the gelatinous material contained in the loculi was the result of mucoid degeneration of the sarcomatous elements.
Sutton and Aveling then go on to say that the specimen was of great interest from an etiologic standpoint. "Man, in common with other mammals, possesses a persistent pedicle of the allantois, familiar under the name of the urachus. This structure is frequently found dilated into a cyst, usually of small size. An account of these allantois cysts, with reference to a few recorded cases, will be found in the Path. Soc. Trans., xxxvi, 523." They drew attention to the fact that Mr. Lawson Tait, in his work on Diseases of the Ovaries, had described certain growths which he regarded as probably originating in the urachus, and which attained such considerable dimensions as to require operative interference.
They thought that, in the present case, they had to deal with an allantois cyst, the walls of which had become sarcomatous, thus affording another illustration of the great tendency exhibited so often by aberrant and ill-developed structures to become the seat of morbid growths, such as sarcoma or carcinoma.
[After a somewhat careful study of the literature on the subject of umbilical tumors, the interpretation of Bland-Sutton and Aveling is not altogether clear. It would rather seem as if we are dealing with a myoma. The gross description speaks of non-striped muscle, and this the histologic picture substantiates. The gross and histologic appearance of the nodule coincides with the appearances presented by uterine myomata. The areas that were supposed to be sarcomatous and inclosed cavities presenl ing a m ucoid appearance might very readily have been due to hyaline degeneration. Without an opportunity of examining their specimen we should hesitate to express any definite opinion as to this case, further than that their interpretation does not seem to tally with the recorded cases of secondary growths attributed to the allantois. — T. S. C]
Doran* says that Aveling and Bland-Sutton had already reported a case of multilocular myxosarcoma of the sheath of the urachus, but it did not involve the urachal canal, and was quite unconnected with the bladder. The specimen (No. 417 b) in the pathologic series of the Museum of the Royal College of Surgeons of England was supposed, when first examined, to have developed in the urachus, but Mr. J. H. Targett considered that it was a myxosarcoma which had originated in the connective tissue surrounding the bladder.
Aveling: Brit. Gyn. Jour., 1886-87, ii, 56 and 187. t Doran, Alban H. G. : The Lancet, 1909, i, 1304.
After I had made my comment on Aveling and Bland-Sutton's case, Alban Doran's note on the case came to my notice, clearly showing a lack of unanimity of opinion among those who had examined the specimen, not only as to the exact character of the tumor, but also as to its precise source of origin.
Fig. 257. — A Multilocular and Malignant Cyst of the Urachus. Gyn.-Path. Nos. 10368 and 1048S. The cyst lay between the abdominal muscles and the peritoneum of the anterior abdominal wall. Below it was attached by a pedicle near the top of the bladder. Upward it extended for a considerable distance above the umbilicus. The omentum was densely adherent to its upper surface. The cyst -wall anteriorly was so thin that I cut it, thinking that it was peritoneum. The cyst is composed of one large and many smaller cavities. Projecting into the large cyst are many smaller cysts, and papillary and solid growths spring from the inner surface of the cyst. Some of the smaller cysts have smooth walls, as is well seen in the one near the pedicle of the tumor. Cross-sections of other small cysts show that they are partially filled with secondary growths. It will be noted that the uterus, tubes, and ovaries are absolutely independent of the cystic tumor. They are, however, partially covered over with secondary cancerous nodules. (For the histologic appearances in this case see Figs. 261, 262, 263.)
A Large Multilocular Carcinomatous Cyst of the Urachus; Secondary Growths in the Pelvis
I saw Mrs. W. W., aged thirty-seven, in consultation with Dr. E. S. Mann, of Dallastown, Pa., and had her admitted to the Johns Hopkins Hospital, October 6, 1906. This patient had never been pregnant. Her menses had commenced at fourteen and had always been regular until the previous year. Her last period had occurred sixteen months before admission. About two years before I saw her, she had noticed, on moving, a sharp, sticking pain in the left lower abdomen. For about a year and a half she had had some abdominal enlargement, and eight weeks before admission the abdomen had commenced to swell a great deal. The feet and legs had also been swollen. The patient gave a history of having lost 20 pounds in the past six months. She had had dysuria, and had had to void four or five times during the night.
c Fig. 258. — Giant-cells in the Wall op an Adenocarcinomatous Cyst of the Urachus. (X 90 diam.) Gyn.-Path. Nos. 10368 and 10488. Occupying the center of the field are slit-like spaces lined on one or both sides with giant-colls. The most perfect picture is that seen at a. At 6 is a giant-cell lying in the stroma. From this picture as a whole one gets the impression that these slit-like spaces may be due to the cracking of brittle giant-cells. At c are the epithelial cells lining a gland-like space of the carcinomatous cyst. Scattered throughout the field are quantities of small round-cells. Many of these have absorbed brown pigment, have swollen up, and at first sight look like vacuoles. In the center of these pale round or oval spaces the small round, deeply staining nucleus is still clearly visible. At d the stroma has undergone almost complete hyaline transformation.
On admission it was noted that she was a well-nourished woman, weighing 172 pounds. The abdomen was markedly distended. It rose rather abruptly from the symphysis to the umbilicus, and then gradually shaded off to the xiphoid. On percussion fluid was evident in all parts of the abdomen. About two months before she had noticed large and small lumps in various parts of the abdomen. Some of these were fully an inch in diameter, and they had sharp edges.
Fig. 259. — Giant-cells in the Wall of an Adenocarcinoma of the Urachus. ( X 90 diam.) Gyn.-Path. Nos. 10368 and 104SS. At a is a slit-like space lined on both sides with a large giant-cell. The nuclei of the giant-cells are irregularly distributed and stain deeply. Extending from one end of the space to the other is a delicate strand. This, under a higher power, was found to contain two small nuclei. At b is an irregular oblong space with a large giant-cell in the center of its upper margin, and an irregular mass of protoplasm containing numerous nuclei bordering its lower margin; projecting into the cavity from either end are delicate filaments of stroma devoid of nuclei. At c is a series of parallel slits. The tissue at this point consists of hyaline material. Most of these slits have no lining whatsoever, but both the upper and lower slit have small giant-cells attached to their margins. At d is a slit-like space lined with giant-cells, e is a giant-cell that could be clearly focused at another level. It was irregularly triangular in shape, and contained a quantity of oval, uniformly staining nuclei arranged chiefly at one end of the cell. There were other giant-cells scattered throughout the field. The protoplasm of some of these was brownish in color, apparently owing to the absorption of old blood-pigment. The stroma of the cyst-wall in this region consisted of fibrous tissue. In the vicinity of these giant-cells and in the neighborhood of the slit-like spaces it showed a great deal of hyaline trans formation; many of the small round-cells that still persisted were swollen and contained a yellowish or brownish pigment — undoubtedly caused by old hemorrhage.
On pelvic examination the cervix was found to be perfectly normal; nothingfurther could be made out.
Operation (October 8, 1906). — On opening the abdomen I immediately came in contact with the contents of a cyst. This cyst was large, multilocular, and intimately adherent to the anterior and lateral abdominal walls (Fig. 257). At first I thought it was impossible to remove it, but on continuing the incision upward we entered the general peritoneal cavity. I then delivered the tumor from above downward. Its pedicle sprang from the top of the bladder. This pedicle was 1 cm. broad and 2 mm. thick. Raw areas were left, both on the anterior and lateral abdominal walls. The bleeding was checked by sliding over the peritoneum as far as possible, thus bringing the raw areas together and diminishing the size of the denuded space.
Both ovaries were normal in size, but were somewhat glued down to the pelvic floor. As the pedicle of the cyst sprang from the bladder, I thought it advisable to turn it in, fearing that there might be an opening between the bladder and the cyst. In the pelvis were metastatic deposits, some of them very minute, others irregular, somewhat translucent, and fully 1 cm. in diameter. The appendix was removed, and the abdomen closed. The patient was discharged November 5, 1906. In answer to an inquiry Dr. Mann wrote me that the patient died January 8, 1908.
Gyn.-Path. Nos. 10368 and 10488.— The cyst-walls vary considerably in thickness. At some points they are thin and transparent; at others they reach the thickness of about 2 cm. These solid areas also contain cysts, and in the small cysts is a blackish-colored fluid. The entire specimen is vascular, and in some places friable and apparently malignant.
On histologic examination the walls are found to consist in part of fibrous tissue, with a definite laminated arrangement. In many places necrosis has taken place, and the tissue presents a homogeneous appearance or takes the stain very poorly. At other points in the walls the connective-tissue cells have taken up much brown pigment, evidently from a long-standing hemorrhage. Here and there throughout the walls are slit-like spaces, the smaller ones surrounded by giant-cells * (Fig. 258) . The giant-cells really consist of large masses of protoplasm containing oval or round, deeply staining nuclei (Fig. 260), and some of these nuclei are four or five times the size of the surrounding ones. Where the cavities are larger, giant-cells may be seen clinging to one side of the cavity, other portions of the cavity being devoid of a lining (Fig. 259). At certain points are aggregations of giant-cells, and interspersed are small, slit-like spaces. One is instantly reminded of the giant-cells and slit-like spaces noted by Bondi, and on careful examination we found here and there crystals lying in the cavity, such as were also found by Bondi. Other portions of the tumor show gland-like spaces lined with one or more layers of epithelium (Fig. 261). The nuclei of the epithelial cells are oval and vesicular, or are deeply staining, and the epithelium itself is of the low cylindric variety. In some places the epithelium has proliferated to a moderate extent. The gland arrangement in some places suggests a papillary formation (Fig. 262), and the gland cavities are filled with a homogeneous material that takes the eosin stain. The epithelial cells at other points are almost flat. There does not seem to be much variation in the size of the cells, and such a picture alone would suggest a papillocystoma. At other points the epithelium has proliferated markedly, so that we have what appears to be solid nests; or the epithelium has melted away, as is noted in colloid carcinoma.
I am fully aware of the frequency with which foreign-body giant-cells are prone to occur in the walls of certain cysts and elsewhere, but the giant-cells in this case are rather unusual, hence I have described them more or less in detail.
Fig. 260. — Giant-cells in the Wall of an Adenocarcinomatous Cyst of the Ukachus. ( X 90 and 300 diam.) Gyn.-Path. Nos. 10368 and 10488. A. a seems to be a large, gland-like space filled with coagulated blood and exfoliated epithelium. It is lined with one layer of low cuboid epithelium, well seen at b. c is a large blood-vessel. Scattered throughout the stroma of the cyst-wall are giant-cells and quite a number of slit-like spaces lined with giantcells. Traversing the slit-like spaces (d) are delicate strands, one of which contains very small nuclei.
B. This shows an enlargement of the oblong area in A. The stroma consists of fibrous tissue. At a is a nest of cancer-cells which has retracted from the surrounding connective tissue. 6 is a deposit of calcareous material near the wall of a blood-vessel, c and d are slit-like spaces, c is lined with a ribbon of protoplasm showing nuclei scattered fairly evenly throughout it. It is impossible to detect any division of the protoplasm into individual cells. The space d is lined with a wide zone of protoplasm showing many nuclei, uniform in size and staining properties, equally distributed throughout the protoplasm, e is another slit-like space lined with a ribbon of protoplasm containing only a single row of nuclei. 42
Fig. 261. — Adenocarcinoma op the Urachus. ( X 90diam.) Gyn.-Path. Nos. 10368 and 10488. The growth at this point bears considerable resemblance to a papillocystoma of the ovary; it consists of large and small irregular spaces lined almost exclusively with one layer of cuboid or low cylindric epithelium, a is a very good example of one of the spaces with a projection into it from the side. This space is lined with one layer of cuboid epithelium containing relatively round and deeply staining nuclei. These nuclei are particularly well seen at 6. The granular contents in the gland-spaces consist of coagulated epithelial secretion. The epithelial elements in the left lower part of the picture have to a large extent melted away. The fibrous stroma of the growth contains very few nuclei, c is one of the blood-vessels in the stroma. From this picture alone one could not tell definitely whether the growth was malignant or not. That it is malignant, however, is definitely settled by a reference to Kig. I'll:',, and also by the fact that at operation metastases were found.
There is no doubt we are dealing with a multilocular cyst that has become malignant. This cyst certainly belongs to a rare type. Of the malignancy, there can be no doubt, because metastases in the pelvic peritoneum were noted at operation (Fig. 263). It did not spring from the ovaries, as they were perfectly normal in size and distant from the growth. Its pedicle, as noted from the history, sprang from the top of the bladder. It will further be noted that during the removal of the tumor a large part of the peritoneum of the anterior and lateral abdominal walls had to be sacrificed. This tumor evidently originated from the urachus.
Fig. 262. — A Papillary- like Area in an Adexocarcixomatous Cyst of the Urachus. (X 90 diam.) Gyn.-Path. Nos. 10368 and 10488. The picture is a rather confused one. At a the complex papillary mass is seen covered with one layer of cuboid epithelium having round, uniformly staining nuclei. At b are two definite glandlike spaces. At c is a bluntish projection of the stroma into a gland-space, d indicates the stroma, consisting of spindleshaped connective-tissue cells. The gland-spaces are filled with a granular, homogeneous material seen at e. (For the appearances of the metastases see Fig. 263.)
The mode of origin of the giant-cells has been of especial interest to me. It will be noted that these giant-cells have been found almost entirelv in the outer connective-tissue wall of the large cyst, and that the cavities that they line are slitlike. This is particularly well seen in Fig. 258. Furthermore, in the vicinity of these slit-like spaces are well-formed giant-cells lying completely surrounded by stroma (Fig. 258, b). On examining the space b in Fig. 259, one gathers the impression that the tissue has been especially brittle, and that during the process of hardening the giant-cells may have split lengthwise; this impression is still further strengthened by examining the area c in Fig. 259. Here the protoplasm has apparently been split up into several long strands. At the upper end of this area there is an intact giant-cell. The finer structure of the giant-cell is well seen in Fig. 260, B, d.
Fig. 263. — Metastasis from Adenocarcinoma of the Urachus. ( X 90 diam.) Gyn.-Path. Nos. 10368 and 10488. o and a are blood-vessels. Scattered throughout the field are nests of epithelial cells. Although originally the growth was glandular, the metastases have tended to form solid nests. At 6, however, two gland-like spaces can be faintly made out. During the process of hardening the cancerous tissue tended to retract from the stroma. This is especially well seen at c. The stroma of the growth showed considerable small-round-cell infiltration.
In an examination of a large number of ovarian cysts I have never seen a picture analogous to the one here depicted. To be sure, in very young dermoid cysts of the ovary, giant-cells are the rule, but here they are invariably lining or clinging to the walls of small cysts — such giant-cells are the embryonic stages of squamous epithelium.
Dr. William H. Welch informed me that he had occasionally seen giant-cells similar to these in the walls of cysts and elsewhere, and suggested that they might be foreign-body giant-cells. He further suggested the possibility of their developing around crystals. On careful examination of many giant-cells I found just one crystal. This was irregular in form. Whether the giant-cells in this case are foreign-body cells or not I cannot say. This point, of course, is of interest only to the pathologist.
Bondi reported a small umbilical cyst of unknown origin. He found quantities of giant-cells analogous to those here depicted (Fig. 266), and in his case some of the giant-cells surrounded crystals. Although his cyst was not malignant, it is of such interest in connection with my case that I shall here report it somewhat in detail.
A Rare Umbilical Cyst
Bondi* reports this case from Schauta's clinic. The patient was a woman, sixty-two years of age. She had had three normal labors. About twenty months before coming under observation she noticed that the umbilicus was larger than usual, and that the abdomen had increased in size. She had never noticed a tumor projecting outward beyond the level of the umbilicus.
Fig. 264. — An Umbilical Cyst. (After Bondi.) The original tumor was 5 cm. in diameter. The drawing has been made from the hardened specimen, which was much contracted. Nearly two years before operation the patient had noted an enlargement at the umbilicus. The overlying skin was brownish in color, tense, and elastic. It was slightly compressible. H is the skin covering the cyst; Nr, the confines of the umbilical depression; P, a prolongation of the peritoneal cavity into the mass. The walls of the cyst were composed of two layers — an outer, consisting of whitish tissue, and an inner, homogeneous zone, grayish brown in color. The cyst contents were spongy, yellowish brown, and soft. (For the histologic picture see Figs. 265 and 266.)
Fig. 26.5. — Wall of an Umbilical Cyst. (After Bondi.) This is a section of the cyst-wall seen in Fig. 264. H represents the skin, with connective tissue immediately beneath it ; B, a dense layer of connective tissue. Rx, granulation tissue. In this are areas containing small spaces. These spaces, as seen in Fig. 266, are lined with giant-cells. The cells in this layer contain blood-pigment. The inner surface (F) consists of coarse and fine threads of fibrin.
At operation, at the umbilicus was a tumor 5 cm. in diameter, the skin over it being brownish in color. It was tense and elastic, showed no marked fluctuation, and was slightly compressible. The abdominal enlargement was due to a multilocular ovarian cyst the size of a man's head, with torsion of the pedicle to the extent of 180 degrees; the wall of the cyst was partially necrotic.
Bondi, J.: Zur Kasuistik der Nabelcysten. Monatsschr. f. Geb. u. Gyn., 190.5, xxi, 729.
646 THE UMBILICUS AND ITS DISEASES.
In the hardened specimen the umbilical cyst was 2.5 cm. in diameter. It lav over an outward prolongation of the abdominal cavity, much as a cap
would fit (Fig. 264). The walls of the
.v^^T^^- c y s ^ nac * two layers, the outer consist ■ > .'! "•' <. j n g f whitish tissue 2 mm. thick. It
V, ' /Sl'Vi* y&SZ?' "I "".s*' "' was adherent to the skin and to the
." 'Mi' 1 ^,^ *is*» peritoneum, and the inner zone consisted \ ii ^ v -F~'~' u; $?l£'Z •^N;* of a broad, homogeneous, gray-brown H%» . -, - : ^ tissue. The cyst contents were spongy, % %\Sj ! /<?^j.- M ?^ ,; l||-: yellowish brown, and soft. Its length } » ;f ' "^/v^C — *^^ in the hardened specimen was 2.5 cm., ^ l*^/j§^- '^" "<#^ : f and its greatest thickness, 1.5 cm. The v\'%fj|&/^/; )' ^,;f.-."--^'* *£• V outer wall of the cyst consisted of fibrous ,Ui ^ ^-^^^'v^^ tissue, which gradually passed over into s^- •*'-'* v *|^' the inner, homogeneous lining, consist'* V *'^ ;* 'o» '"■* ing of young fibrous tissue. This gradu"*%t£ j , v-** ally merged into the granulation tissue
" **- bx which lined the cavity. The granulation fig. 266.— Giaxt-cells in the Wall of an u.mbili- tissue here and there contained blood-pigon i.) ment. Here and there near the inner
Scattered throughout the inner wall of the cyst
(Fig. 26.5) were aggregations of small, siit-iike spaces. surf ace were numerous spaces, often oc Some of these are lined with one layer of epithelium, CU lTmg ill groups. These Were regularly
others with giant-cells. The nuclei of the giant-cells .... ,-,-,. __ _ . o^^x
are uniform and fairly evenly distributed throughout lmed With giailt-CellS (t lgS. 265 and 266) .
the protoplasm. j n t nese spaces were crystals showing that
the spaces were not artefacts. Bondi says that it was not a dermoid, but a peritoneal cyst, into which a hemorrhage had occurred.
It is possible that these giant-cells were foreign-body giant-cells. As already pointed out, they bear a marked resemblance to those noted in the malignant cyst of the urachus I have just recorded so fully. (See Figs. 258, 259, and 260.)
Consulted On Malignant Growths Of The Urachus And Urachal Region.
Aveling: Brit. Gyn. Jour., 1886-87, ii, 56, 187.
Bondi, J.: Zur Kasuistik der Nabelcysten. Monatsschr. f. Geb. u. Gyn., 1905, xxi, 729.
Doran, A.: Stanley's Case of Patent Urachus with Observations on Urachal Cysts. St. Bartholomew's Hospital Reports, 1898, xxxiv, 33.
Doran, A. H. G.: Urachal Cyst Simulating Appendicular Abscess; Arrested Development of Genital Tract; with Notes on Recently Reported Cases of Urachal Cysts. The Lancet, 1909, i, 1304.
Fischer, H.: Die Eiterungen im subumbilicalen Raume. Volkmann's Sammlung klin. Vortrage, N. F., No. 89 (Chir. No. 24), Leipzig, 1894, 519.
Frank, T.: Zur Casuistik der Urachustumoren. Inaug. Diss., Wurzburg, 1893.
Graf, F.: Urachusfisteln und ihre Behandlung. Inaug. Diss., Berlin, 1896.
Hoffmann, C. E. E.: Zur pathologisch-anatomischen Veranderung des Harnstrangs. Arch. der Heilkunde, 1870, xi, 373.
Koslowski, B. S. : Ein Fall von wahrem Nabeladenom. Deutsche Zeitschr. f. Chir., 1903, lxix, 469.
Rotter: Blasencarcinom kombinirt mit Urachuscyste. Centralbl. f. Chir., 1897, xxiv, 604.
Wolff, C. C. : Beitrag zur Lehre von den Urachuscysten. Inaug. Diss., Marburg, 1873.
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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