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Purpose: Enteric type adenocarcinomas arising in the dome of the bladder or along the urachal ligament are uncommon. To improve our understanding of urachal carcinoma and define outcome with current management, we performed a retrospective review of cases seen at the M. D. Anderson Cancer Center., Materials and Methods: We reviewed the records of 42 patients with urachal carcinoma evaluated at our institution from 1985 to 2001. Specifically, we sought to evaluate the importance of extent of disease, surgical characteristics and systemic therapy on clinical outcome., Results: Of the 42 patients 7 had clinically evident metastases at diagnosis and 35 had resectable disease that was managed initially with surgery. Overall survival from diagnosis for all 42 patients was 46 months with 40% surviving at 5 years. Of the resected cases 16 (46%) remain disease-free (median followup 31 months). Covariates associated with long-term survival were negative surgical margins (p = 0.004) and absence of nodal involvement (p = 0.01). Median survival from recognition of metastatic disease was 24 months in 26 patients in whom metastases ultimately developed. Chemotherapy for metastatic disease produced only 4 significant responses, including 3 of 9 patients treated with 5-fluorouracil and cisplatin containing regimens., Conclusions: Urachal carcinomas are usually locally advanced at presentation with a high risk of distant metastases. However, long-term survival following radical resection occurs in a significant fraction of patients (16 of 35 in our series), supporting an attempt at margin-negative, en bloc resection if at all possible. Chemotherapy appropriate for enteric type adenocarcinoma can induce objective responses but meaningful improvement in survival is not yet demonstrated.

Purpose: Primary adenocarcinoma of the bladder is a rare neoplasm whose histogenesis is poorly understood. Current data support the concept that adenocarcinoma of the bladder and urachus evolves from zones of intestinal metaplasia that become dysplastic and invasive. To address this hypothesis further we determined the immunoreactivity of benign and malignant epithelial tissue from the bladder and urachus with a monoclonal antibody that is reactive with colonic epithelium to evaluate the presence of a common reactive epitope., Materials and Methods: The monoclonal antibody 7E12H12 (IgM isotype), developed against a colonic epithelial protein, was used in an immunoperoxidase assay to survey formalin fixed, paraffin embedded archival tissue specimens. A total of 26 specimens obtained by endoscopic biopsy or extirpative surgery, including benign and malignant bladder and urachal epithelial abnormalities, was chosen for retrospective evaluation., Results: All adenocarcinoma reacted positively regardless of the histological variant, differentiation, or bladder or urachal origin. In contrast, transitional cell and squamous cell carcinomas were nonreactive. Also, the pattern of reactivity in tissues that contained benign epithelial proliferations suggested a stepwise transition with no reactivity in normal urothelium or Brunn's epithelial nests, rare staining of cystitis cystica, and uniformly positive reactivity in cystitis glandularis and frank colonic intestinal metaplasia of the bladder and urachus., Conclusions: The shared, aberrant phenotypic expression of a unique colonic epitope in benign epithelial metaplasia, and adenocarcinoma of the bladder and urachus suggests a common underlying pathway toward adenocarcinoma in cystic and urachal adenocarcinoma. The implications for diagnostic pathology are discussed.

We studied the structure and histologic appearance of intramural tubular urachal remnants in bladders from an unselected series of 122 autopsies, in which the patient age at death was 38 to 91 years. The bladders were fixed with formaldehyde introduced 10 minutes to 5 hours post mortem. Of the bladders 32 per cent showed tubular urachal remnants. The remnants were classified into 3 types on the basis of the epithelial extent and structure. Of the remnants 68.4 per cent had lumen covered with transitional epithelium, while the remainder had columnar epithelium. Bud-like epithelial proliferations were found in 43.6 per cent and mild dysplasia of the epithelium was found in 7.7 per cent of the urachal remnants, while mild inflammation in the loose peritubular tissue was demonstrated in 23.1 per cent.

Urachal cancers are uncommon malignancies with a location that often permits considerable local extension before they are discovered. The most common histological type is adenocarcinoma, which may produce mucus that is a valuable aid in diagnosis. The presence of stippled calcification in a midline abdominal wall mass is almost pathognomonic for urachal carcinoma. More commonly, however, the symptoms are less specific, such as hematuria and an abdominal mass. Many lesions are visible endoscopically and, thus, the diagnosis can be made preoperatively from a biopsy. Most treatment failures occur because the tumor is not controlled locally by the initial operation and, therefore, we recommend en bloc cystectomy with umbilectomy and pelvic lymphadenectomy unless the tumor is known to be a sarcoma or early stage (I) carcinoma. If these patients are undertreated and there is a local recurrence then the patient usually is not salvageable. Because of the difficulty in identifying the origin of a bladder adenocarcinoma, any tumor on the dome or anterior wall should be approached initially as if it were a urachal tumor.

A case of mucin-producing adenocarcinoma of the urachus is reported. Preoperative diagnosis was established by computerized axial tomography. The embryology, histogenesis, signs, symptoms, diagnostic criteria and treatment are discussed.

The third documented case of primary squamous cell carcinoma of the urachus in the English literature is reported. The interesting coincidental occurrence of the dermoid cyst of the right ovary and a complete longitudinal septum of the vagina and uterine cervix is described.

During the last 50 years 8 patients with adenocarcinoma of the urachus were managed at our hospital. The various modalities of therapy included radiation therapy, transurethral resection, segmental resection of the bladder and total cystectomy. A retrospective analysis of these cases is presented, including the histologic patterns, sites of spread and survival according to therapy.

After 2 years of mucusuria a villous adenoma of the urachus was resected from a woman by partial cystectomy and excision of the entire urachus. Convalescence was uneventful and the patient was well 12 months later. Urachal adenomas are rare, this being the sixteenth case reported. Generally, they are multilocular cystic tumors lined by columnar epithelium with a population of mucous goblet cells. Often, there is a striking resemblance to gut epithelium. Ultrastructurally, by transmission and scanning electron microscopy, the tissue resembles gut mucosa. Urinary mucus is a common and relatively specific symptom for adenomas of the lower urachus, occurring in 7 of 11 cases. Resection alone is effective therapy but care must be exercised to avoid spilling adenoma cells in the peritoneum.