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Diseases associated with persistent urachus are relatively rare. During the past 9-year period, there have been 14 patients with urachal disease consisting of 10 with urachal abscess and 4 with urachal cancer. The 10 patients with urachal abscess consisted of 7 males and 3 females aged 19-77 years (mean, 46 years). The 4 patients with urachal cancer consisted of 2 males and 2 females aged 48-81 years (mean, 57 years). As symptoms, lower abdominal pain was frequently observed in the patients with persistent urachus with abscess and gross hematuria in those with urachal cancer. Echo and magnetic resonance imaging (MRI) were useful for visualizing the lesion. Computed tomogtaphic (CT) scanning could not visualize the lesion in 2 patients. Nine patients underwent MRI, which visualized the lesion in all of them. As urachal abscess, an umbilical fistula was observed in 3 patients, urachal cyst in 4, and urachal diverticulum in 1. The preoperative diagnosis was urachal cancer in 6 patients, and pathological examination showed 4 patients with adenocarcinoma, 1 with inflammatory granuloma, and 1 with pseudosarcoma. Urachal abscess was treated by resection of the abscess in 6 patients, transurethral resection in 1, and resection of the umbilicus and urachus and total cystectomy in the other. Of the patients with urachal cancer, 1 underwent total cystectomy and the other 3 underwent total urachal resection and partial cystectomy. In 2 patients with persistent urachus with abscess, the differentiation between abscess and malignant tumor was difficult.

A 47-year-old female consulted our hospital with the chief complaints of lower abdominal pain and fever. There was a palpable mass in the lower abdomen. The patient had undergone oophorectomy by lower abdominal median incision. Ultrasonography, computed tomography, and magnetic resonance imaging (MRI) demonstrated a cystic mass above the bladder dome extending to the umbilicus, which was strongly suspected to be a urachal tumor. Enhanced T1 weighted MRI showed a mass enhanced by contrast media. Partial cystectomy with urachal resection was performed by lower abdominal median incision. The histological diagnosis was xanthogranuloma. This is the thirteenth case report of xanthogranuloma of the urachus in the Japanese literature.

We report a case of transitional cell carcinoma of urachus in a 72-year-old man. At follow up cystoscopy for past history of bladder cancer, we found a papillary tumor in the right orifice that came out to the bladder cavity intermittently. Although there was no cancerous lesion on the surface of the bladder mucosa, a submucosal eminence at the dome of bladder was observed. Sagittal magnetic resonance imaging (MRI) revealed an extravesical tumor (2 cm) at the position of urachus. Under the diagnosis of right ureteral cancer and urachal cancer, we performed right distal ureterectomy, ureteral reimplantation and total resection of urachus. Pathological examination revealed transitional cell carcinoma in the urachus and right ureter. The urachal cavity was isolated completely from the bladder cavity. Tumor infiltrated to the muscularis of the bladder dome from the urachal cavity, but there was no cancerous lesion on the surface of the bladder mucosa. Therefore, our diagnosis was urachal transitional cell carcinoma and right ureteral carcinoma.

A 31-year-old man was referred to our hospital for evaluation of urachal rest. The history of his present illness dated back to birth, when the umbilicus was projected and urinary discharge was noted. At that time, the symptom of discharge had spontaneously subsided. At the age of 22, however, the patient again experienced discharge from the umbilicus. Although he did not seek treatment, after six years this symptom disappeared. Around this time, however, pyuria was revealed during medical examination, and abdominal ultrasonography (US) suggested the presence of urachal rest. At the time of hospitalization, physical examination revealed that the patient's right testis was not palpable. He was diagnosed with patent urachus with hemilateral aplasia and monorchism by US, computed tomography, magnetic resonance imaging and cystoscopy. The patient subsequently underwent radical operation. Patent urachus in adults is very rare, and only a few cases have been reported. To our knowledge, only one previously reported case involved a recurrence after spontaneous healing. Further, this is the first report of a patient with patent urachus with hemilateral aplasia and monorchism. Radical operation is generally recommended, based on the fact that very few cases heal conservatively.

A case of an early urachal carcinoma is reported. A 46-year-old man was referred to our hospital because of urinary bladder tumor. Microhematuria had been detected by a medical health examination two months earlier. Gold punch biopsy under cystoscopy proved adenocarcinoma. Sagittal view in magnetic resonance imaging (MRI) clearly demonstrated a small tumor at the bladder dome. En bloc partial cystectomy and pelvic lymphadenectomy was performed under diagnosis of early urachal carcinoma. Microscopically, papillary adenocarcinoma like colon carcinoma was revealed, but the bladder wall and urachus were free from the infiltration of carcinoma cells and no pelvic lymph node metastasis was found. There has been no evidence of recurrence or metastasis 5 months after the operation.

We report a patient with urachal transitional cell carcinoma invading the sigmoid colon. The diagnostic and therapeutic aspects of this disease are discussed. The literature is also reviewed. This 42-year-old man was admitted with the chief complaint of micturition pain. Operation consisting of total cystourethrectomy, excision of the urachus together with the umbilicus, pelvic lymphadenectomy, ilial conduit plasty, sigmoidectomy, and colorectostomy was performed on June 3, 1986, after the diagnosis of urachal carcinoma invading the sigmoid colon. A tumor the size of a hen's egg was found at the dome of the bladder extending to the sigmoid colon. The urachus 8 cm in length and 2 cm in diameter extended from the site of the tumor to the umbilicus. Histologically the tumor was a transitional cell carcinoma Grade III. The urachus was composed of epithelioid and muscle layers, and its lower end was filled with necrotic inflammatory debris so that the canal was obliterated; tumor cells infiltrated the muscle layer. No metastasis was found in lymphnodes. Combination therapy of 5 fluorouracil, adriamycin and cis-platinum was given after operation. He has returned to work and is doing well without evidence of metastasis or recurrence now at 1 year after the operation.

We report a case of urachal carcinoma with elevated serum carcinoembryonic antigen (CEA) level in a 36-year-old man. En bloc resection was performed. The production of CEA was proved histologically. The level of serum CEA returned to the normal value after operation. Seventeen postoperative months the level of serum CEA was noted to be elevated again, and 2 months later symptoms appeared and tumor recurrence was revealed on computer tomographic scan. Radiotherapy and chemotherapy were done without any noticeable response. He died of tumor progression. In our case, serum CEA gave useful information as a tumor marker. It showed specificity for the tumor and helped evaluation of tumor resection as well as detection of tumor recurrence.

Four cases of urachal carcinoma are presented. The first case was a 36-year-old man who was admitted with the chief complaint of macrohematuria with mucinous debris. Cystoscopic examination revealed a single tumor at the dome of the bladder. Partial cystectomy and postoperative radiation therapy were performed. Histological findings showed a well differentiated adenocarcinoma. He has been well for twelve years postoperatively. The second case was a 52-year-old man who was admitted with the chief complaint of macrohematuria with mucinous debris. Cystoscopic examination revealed a single tumor at the dome of the bladder. Partial cystectomy and postoperative radiochemotherapy were performed. Histological findings showed a moderately differentiated adenocarcinoma. He died of a recurrent tumor 30 months after operation. The third case was 70-year-old man who was admitted with the chief complaint of macrohematuria with mucinous debris. Cystoscopic examination revealed multiple tumors at the dome of the bladder, internal urethral orifice and right lateral wall. Total cystectomy, ureterocutaneostomy and postoperative chemotherapy were performed. Histological findings showed a poorly differentiated adenocarcinoma. He died of a recurrent tumor 30 months after operation. The fourth case was a 68-year-old woman who was admitted with the chief complaint of macrohematuria with mucinous debris. Cystoscopic examination revealed a single tumor at the dome of the bladder. CT scan demonstrated a mass with calcification extending from the bladder dome superiorly. Partial cystectomy and postoperative chemotherapy were performed. Histological findings showed a well differentiated adenocarcinoma. She has been well for three months postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)

A 71-year-old man was admitted with the chief complaint of gross hematuria. Cystoscopic examination showed a broadbased papillary tumor at the apex of the bladder. There was edema of the mucous membrane around the tumor. CT-scan demonstrated a mass extending from the bladder dome superiorly. Partial resection of the bladder was done. Pathological examination revealed grade 3 transitional cell carcinoma, with scattered adenocarcinoma and squamous cell carcinoma foci. Chemotherapy with bleomycin (BLM) and cis-dichlorodiamine platinum (CDDP) was done postoperatively. Ten months after the operation, he was readmitted because of recurrence in retroperitoneal lymph nodes. Chemotherapy with BLM and CDDP was done, but he died of pulmonary complications. Autopsy revealed retroperitoneal lymph node metastasis of transitional cell carcinoma. There was no metastasis to any other organ. We briefly discuss 275 cases of the carcinoma of the urachus collected from the Japanese literature.