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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.