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Mucoid Carcinoma of the Urachus
Department of Case Reports New Instruments, Etc. MUCOID CARCINOMA OF THE URACHUS M.
LEO BOBROW,
M.D., F.A.C.S., NEW YORK, N.Y.
(From the Department of Gynecology, Harlem Ho.spital)
THE paucity
of reports of mucoid carcinoma arising in the urachus is rather striking, since neither tumors of the bladder nor machal rests are particularly rare. Beggl stated that careful histologic search would reveal urachal rests or cysts in the majority of the cases examined. This fact appears to be substantiated by Saphir and Kurland,2 who demonstrated the presence of typical tubular structures in the vault of the bladder in the region of the ligamentum umbilicale mediale in 9 of 10 bladders. ~;hese carcinomas arise from the epithelium of the urachal canal, and are, in reality, not tumors of the bladder at all, but merely masquerade as such after they have invaded the bladder mucous membrane. Begg,s in 1931, collected 44 cases of assorted urachal tumors from the literature, with only 19 reported cases of mucoid carcinoma. He stated that the colloid carcinoma represents primarily a metaplasia of the transitional epithelium followed by a colloid or mucoid degeneration. The sections appear very much like rectal carcinoma. Kalo 14 (1931), Payne and .Jones 15 (1931), Terrier, Craig and Foord,a (1936), Begg,7 (1936), De Waard,s (1939), Mattel,B (1940), and others have added cases of adenocarcinoma of the urachus to Begg's original list. Hughes and La Towskylo (1942) reported 2 cases of mucoid carcinoma of the urachus. Most of those reported had involvement of the bladder mucous membrane. ~;he embryology of the urachus is thoroughly described by Cullen.n The allantois is one of the first structures differentiated in the embryo and is recognized as a recess of the yolk sac extending into the body stalk. In an embryo 2.5 em. long, a portion of the yolk sac has been definitely differentiated into the digestive tract. Its caudal portion has been carried downward and forward, terminating in the cloaca. The allantois now starts :from the cloaca and, after curving upward, passes outward in the body stalk to end in a bulbous extremity near the placenta. By the time the embryo is 7 em. long, the allantois has been partially separated from the bowel by the urorectal septum. The lower portion of the allantois has been convE,rted into the bladder, and its upper part now passes off the fundus, extending upward and forward, passing out in the cord. Prom the foregoing it can be seen that the allantois develops from the yolk sac, that it is one of the earliest structmes to make its appearance, and that, after a time, it is carried downward and forward to the cloaca. The bladder develops from the lower portion of the allantois. The upper portion of the allantois is continued upward to the umbilicus and then into the cord. The intra-abdominal portion is called the urachus. :rhe urachus and allantois soon become solid cords in part of their course, but show an inherent tendency to develop spindlelike dilations at regular intervals. These dilations are characteris6c of the urachus and allantois, and may be found at any period in the development of the embryo. These dilations may persist in many persons, and account for small cysts not infrequently noted at operation. The urachus may occasionally remain
909
910
.\m . .f. Oblt. & Gynec. April, 1953
BOBROW
patent at birth, a nd t his is attested by the number of children born with a urinary umbilical :fistula. Age Incidence.- In Begg'sa series, no tumor occurred in a patient younger than 29 1 and the majority appeared after 50 years of age. In the same series, 80 per cent of the tumors occurred in men. This case is being reported because the mucoid carcinoma was grossly limited to the urachus and the bladder mucous membrane was not involved. P. A., a 42-year-old married para 0, gravida i, entered the hospital on June 21 1952, complaining of severe intermittent lower abdominal pain for 8 days, menorrhagia for 6 months, and frequency of urination. The patient had been told 4 years previously that she had a fibroid uterus which should be removed. Recently, the patient noticed a mass in the lower abdomen that she had not felt previously. Past history revealed that the patient had had malarial fever in 1938. She had a spontaneous abortion at 4 months' gestation in 1927. Her last menstrual period was on May 28, 1952.
F ig·. 1.
Fig. 1.-Mucoid carcinoma of the Fig. 2.-Normal bladder tissue.
F ig. 2. ·~ rachu s.
General physical examination was negative except for blood pressure of 160/90. 1'he abdomen was distended by a mass in the right lower quadrant near the midline, rising up to 4 em. below the umbilicus. There was mild tenderness in the left lower quadrant. Pelvic examination showed the uterus to be enlarged to the size of a 3 months' gestation. The mass, felt abdominally, seemed to be anterior and to the right of the uterus. It was thought to be a subserous nodule. 'l'here were no adnexal masses or tenderness. Rectal examination was negative except for external hemorrhoids. A provisional diagnosis of fibromyomas of the uterus was made. A laparotomy was done on June 61 1952. Operative findings were: an encapsulated tumor 10 em. in diameter, attached to the anterior abdominal wall by a :fiat, fibrous stalk about 4 em. wide. At its upper end, the mass tapered off into the urachus about 2 em. below the umbilicus. The lower end of this tumor was broadly attached to the dome of the bladder. The tumor was filled with degenerated, gelatinous material. The uterus was enlarged to 12 by 10 em. and was nodular. The right ovary measured 6 em . in diameter. On sectioning, it was seen that most of the ovary formed a fibroma, the rest was cystic. The left ovary was cystic. Both tubes were grossly normal. The tumor mass, including the fundus of the bladder,
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MUCOID CARCINOMA OF URACHUS
911
was removed. The bladder was closed in two layers. A total hysterectomy, bilateral salpingo· oophorectomy, and appendectomy were done. The patient's postoperative course was uneventful; an indwelling catheter was main· tainE1d for 9 days. Pathologic Eeport.-Multiple fibromyomas of the uterus; proliferative endometrium; fibroma of right ovary; normal tubes and cervix; encapsulated mucinous-cell adenocarcinoma attached to the wall of the bladder, probably arising in a urachal rest. In order to rule out malignancy of the gastrointestinal tract, a gastrointestinal series and a proctoscopic examination were done on the eleventh postoperative day. Both were nega· tive. 'I'he patient was discharged on the twelfth postoperative day. The wound was healed. Abdominal and pelvic examinations did not show any masses, induration, or tenderness. Microscopic Eeport.-The entire specimen was very friable and contained brownish, mucoid material. Histology: The wall consisted in part of :fibrous tissue with a laminated appearance. In many places, necrosis had taken place and the tissue presented a homogeneous appearance and took the stain poorly. Portions showed glandlike spaces lined with one or more layers of low columnar epithelium exhibiting mucinous secretion. The nuclei of the epithelial cells were oval and vesicular, and deeply stained. In some areas, the epithelium had proliferated to some degree (Fig. 1). Comment.-There is no doubt that we were dealing with a multilocular cyst of the ura· chus that had become malignant. 'l'his growth was not intraperitoneal, and was not connected with anything in the peritoneal cavity. Its pedicle sprang from the top of the blad· der. During the removal of the tumor, a large part of the peritoneum and transversalis fascia of the anterior abdominal wall had to be sacrificed. The portion of the fundus of the bladder adjacent to the tumor was removed to make sure that we were not dealing with a carcinoma of the bladder (Fig. 2). The mucous membrane of the bladder was not involved. This tumor evidently originated in the urachus. The treatment is surgical, and the operative specimen should include the umbilicus, a wedge-shaped block of transversalis fascia, the peritoneum, and at least the fundus of the bladder. I wish to thank Dr. Solomon Weintraub for his pathological study of this ease.
References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11.
Begg, R. C.: J. Anat. 64: 170, 1930. Saphir, 0., and Kurland, S. K.: Urol. & Cutan. Rev. 43: 709, 1939. Begg, R. C.: Brit. J. Surg. 18: 422, 1931. Kalo, A.: Ztschr. f. urol. Chir. 32: 239, 1931. Payne, R. L., and Jones, R. D.: Virginia :M. Monthly 58: 90, 1931. 'I'errier, P. A., Craig, L. C., and Foord, A. G.: Urol. & Ctan. Rev. 40: 769, 1936. Begg, R. C.: Brit. J. Surg. 23: 769, 1936. DeWaard, T.: J. Urol. 42: 554, 1939. Mattei, R.: J. de chir. 55: 84, 1940. Hughes, P. B., and La Towsky, L. W.: Am. J. Surg. 58: 422, 1942. Cullen, Thomas Stephen: Embryology, Anatomy and Diseases of the Umbilicus To· gether With Diseases of Urachus, Philadelphia, 1916, W. B. Saunders Company, pp. 16-23.
LEO BOBROW,
M.D., F.A.C.S., NEW YORK, N.Y.
(From the Department of Gynecology, Harlem Ho.spital)
THE paucity
of reports of mucoid carcinoma arising in the urachus is rather striking, since neither tumors of the bladder nor machal rests are particularly rare. Beggl stated that careful histologic search would reveal urachal rests or cysts in the majority of the cases examined. This fact appears to be substantiated by Saphir and Kurland,2 who demonstrated the presence of typical tubular structures in the vault of the bladder in the region of the ligamentum umbilicale mediale in 9 of 10 bladders. ~;hese carcinomas arise from the epithelium of the urachal canal, and are, in reality, not tumors of the bladder at all, but merely masquerade as such after they have invaded the bladder mucous membrane. Begg,s in 1931, collected 44 cases of assorted urachal tumors from the literature, with only 19 reported cases of mucoid carcinoma. He stated that the colloid carcinoma represents primarily a metaplasia of the transitional epithelium followed by a colloid or mucoid degeneration. The sections appear very much like rectal carcinoma. Kalo 14 (1931), Payne and .Jones 15 (1931), Terrier, Craig and Foord,a (1936), Begg,7 (1936), De Waard,s (1939), Mattel,B (1940), and others have added cases of adenocarcinoma of the urachus to Begg's original list. Hughes and La Towskylo (1942) reported 2 cases of mucoid carcinoma of the urachus. Most of those reported had involvement of the bladder mucous membrane. ~;he embryology of the urachus is thoroughly described by Cullen.n The allantois is one of the first structures differentiated in the embryo and is recognized as a recess of the yolk sac extending into the body stalk. In an embryo 2.5 em. long, a portion of the yolk sac has been definitely differentiated into the digestive tract. Its caudal portion has been carried downward and forward, terminating in the cloaca. The allantois now starts :from the cloaca and, after curving upward, passes outward in the body stalk to end in a bulbous extremity near the placenta. By the time the embryo is 7 em. long, the allantois has been partially separated from the bowel by the urorectal septum. The lower portion of the allantois has been convE,rted into the bladder, and its upper part now passes off the fundus, extending upward and forward, passing out in the cord. Prom the foregoing it can be seen that the allantois develops from the yolk sac, that it is one of the earliest structmes to make its appearance, and that, after a time, it is carried downward and forward to the cloaca. The bladder develops from the lower portion of the allantois. The upper portion of the allantois is continued upward to the umbilicus and then into the cord. The intra-abdominal portion is called the urachus. :rhe urachus and allantois soon become solid cords in part of their course, but show an inherent tendency to develop spindlelike dilations at regular intervals. These dilations are characteris6c of the urachus and allantois, and may be found at any period in the development of the embryo. These dilations may persist in many persons, and account for small cysts not infrequently noted at operation. The urachus may occasionally remain
909
910
.\m . .f. Oblt. & Gynec. April, 1953
BOBROW
patent at birth, a nd t his is attested by the number of children born with a urinary umbilical :fistula. Age Incidence.- In Begg'sa series, no tumor occurred in a patient younger than 29 1 and the majority appeared after 50 years of age. In the same series, 80 per cent of the tumors occurred in men. This case is being reported because the mucoid carcinoma was grossly limited to the urachus and the bladder mucous membrane was not involved. P. A., a 42-year-old married para 0, gravida i, entered the hospital on June 21 1952, complaining of severe intermittent lower abdominal pain for 8 days, menorrhagia for 6 months, and frequency of urination. The patient had been told 4 years previously that she had a fibroid uterus which should be removed. Recently, the patient noticed a mass in the lower abdomen that she had not felt previously. Past history revealed that the patient had had malarial fever in 1938. She had a spontaneous abortion at 4 months' gestation in 1927. Her last menstrual period was on May 28, 1952.
F ig·. 1.
Fig. 1.-Mucoid carcinoma of the Fig. 2.-Normal bladder tissue.
F ig. 2. ·~ rachu s.
General physical examination was negative except for blood pressure of 160/90. 1'he abdomen was distended by a mass in the right lower quadrant near the midline, rising up to 4 em. below the umbilicus. There was mild tenderness in the left lower quadrant. Pelvic examination showed the uterus to be enlarged to the size of a 3 months' gestation. The mass, felt abdominally, seemed to be anterior and to the right of the uterus. It was thought to be a subserous nodule. 'l'here were no adnexal masses or tenderness. Rectal examination was negative except for external hemorrhoids. A provisional diagnosis of fibromyomas of the uterus was made. A laparotomy was done on June 61 1952. Operative findings were: an encapsulated tumor 10 em. in diameter, attached to the anterior abdominal wall by a :fiat, fibrous stalk about 4 em. wide. At its upper end, the mass tapered off into the urachus about 2 em. below the umbilicus. The lower end of this tumor was broadly attached to the dome of the bladder. The tumor was filled with degenerated, gelatinous material. The uterus was enlarged to 12 by 10 em. and was nodular. The right ovary measured 6 em . in diameter. On sectioning, it was seen that most of the ovary formed a fibroma, the rest was cystic. The left ovary was cystic. Both tubes were grossly normal. The tumor mass, including the fundus of the bladder,
Volume 65 Number 4
MUCOID CARCINOMA OF URACHUS
911
was removed. The bladder was closed in two layers. A total hysterectomy, bilateral salpingo· oophorectomy, and appendectomy were done. The patient's postoperative course was uneventful; an indwelling catheter was main· tainE1d for 9 days. Pathologic Eeport.-Multiple fibromyomas of the uterus; proliferative endometrium; fibroma of right ovary; normal tubes and cervix; encapsulated mucinous-cell adenocarcinoma attached to the wall of the bladder, probably arising in a urachal rest. In order to rule out malignancy of the gastrointestinal tract, a gastrointestinal series and a proctoscopic examination were done on the eleventh postoperative day. Both were nega· tive. 'I'he patient was discharged on the twelfth postoperative day. The wound was healed. Abdominal and pelvic examinations did not show any masses, induration, or tenderness. Microscopic Eeport.-The entire specimen was very friable and contained brownish, mucoid material. Histology: The wall consisted in part of :fibrous tissue with a laminated appearance. In many places, necrosis had taken place and the tissue presented a homogeneous appearance and took the stain poorly. Portions showed glandlike spaces lined with one or more layers of low columnar epithelium exhibiting mucinous secretion. The nuclei of the epithelial cells were oval and vesicular, and deeply stained. In some areas, the epithelium had proliferated to some degree (Fig. 1). Comment.-There is no doubt that we were dealing with a multilocular cyst of the ura· chus that had become malignant. 'l'his growth was not intraperitoneal, and was not connected with anything in the peritoneal cavity. Its pedicle sprang from the top of the blad· der. During the removal of the tumor, a large part of the peritoneum and transversalis fascia of the anterior abdominal wall had to be sacrificed. The portion of the fundus of the bladder adjacent to the tumor was removed to make sure that we were not dealing with a carcinoma of the bladder (Fig. 2). The mucous membrane of the bladder was not involved. This tumor evidently originated in the urachus. The treatment is surgical, and the operative specimen should include the umbilicus, a wedge-shaped block of transversalis fascia, the peritoneum, and at least the fundus of the bladder. I wish to thank Dr. Solomon Weintraub for his pathological study of this ease.
References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11.
Begg, R. C.: J. Anat. 64: 170, 1930. Saphir, 0., and Kurland, S. K.: Urol. & Cutan. Rev. 43: 709, 1939. Begg, R. C.: Brit. J. Surg. 18: 422, 1931. Kalo, A.: Ztschr. f. urol. Chir. 32: 239, 1931. Payne, R. L., and Jones, R. D.: Virginia :M. Monthly 58: 90, 1931. 'I'errier, P. A., Craig, L. C., and Foord, A. G.: Urol. & Ctan. Rev. 40: 769, 1936. Begg, R. C.: Brit. J. Surg. 23: 769, 1936. DeWaard, T.: J. Urol. 42: 554, 1939. Mattei, R.: J. de chir. 55: 84, 1940. Hughes, P. B., and La Towsky, L. W.: Am. J. Surg. 58: 422, 1942. Cullen, Thomas Stephen: Embryology, Anatomy and Diseases of the Umbilicus To· gether With Diseases of Urachus, Philadelphia, 1916, W. B. Saunders Company, pp. 16-23.