Peritoneal Carcinomatosis of Urachus Origin Treated by Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC): An International Registry of 36 Patients.

PURPOSE: Peritoneal carcinomatosis or pseudomyxoma peritonei from urachus is a rare form of presentation, often diagnosed at an advanced state of tumor burden. Because of its rarity, little is known about its natural history, prognosis, or optimal treatment. We searched a large international multicenter database of peritoneal surface disease to identify cases of peritoneal carcinomatosis of urachus that were treated with cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) at expert centers. The aim is to improve knowledge and understanding of the disease and standardize its treatment. METHODS: A prospective multicenter international database was retrospectively searched to identify all patients with urachus tumor and peritoneal metastases who underwent CRS and HIPEC through the Peritoneal Surface Oncology Group International (PSOGI). Postoperative complications, long-term results, and principal prognostic factors were analyzed. RESULTS: The analysis included 36 patients. After median follow-up of 48 months, median overall survival (OS) was 58.5 months. Three- and 5-year OS was 55.4 and 46.2%, respectively. Patients who underwent complete macroscopic CRS had significantly better survival than those treated with incomplete CRS, with median OS not achieved and of 20.1 months, respectively [95% confidence interval (CI) 4.4-30.5, p < 0.001]. There were no postoperative deaths, and 37.9% of patients had major complications. CONCLUSION: CRS and HIPEC may increase long-term survival in selected patients with peritoneal metastases of urachus origin, especially when complete CRS is achieved.

[Nonsurgical and surgical treatment of an urachal fistula in an alpaca cria. A case report]. / Konservative und chirurgische Therapie der Urachusfistel bei einem Alpaka-Cria. Ein Fallbericht.

Urachal fistula is a neonatal condition. There are two reported forms: a congenital and an acquired form. We describe the case of a 6-hour-old female alpaca cria that was presented with a damp umbilicus and a meconium impaction. Conservative treatment of the urachal fistula with local and systemic medication was unsuccessful after 6 days; therefore, a resection of the umbilicus under general anaesthesia was performed. Reconvalescence was uneventful.

Urachal carcinoma: surgical and chemotherapeutic options.

The urachal ligament is an embryologic remnant connecting the dome of the bladder to the umbilicus via the ligamentum commune. Autopsy series suggest that in approximately a third of subjects, the urachal remnant may persist with tubular or cystic structures. However, tumors of this site are extremely rare. Patients usually present with hematuria and upon imaging, have evidence of a cystic or solid structure in the bladder dome or in the bladder midline. If a biopsy confirms adenocarcinoma, these tumors should be considered an urachal cancer until proven otherwise. Although there are no prospective clinical trials reported to date, large single-institution reports suggest surgical resection with a partial cystectomy and en bloc resection of the urachal ligament with umbilicus as the treatment of choice in the setting of localized disease. Although there is currently no definitive role for neoadjuvant or adjuvant chemotherapy in this tumor, risk factors predicting progression may allow for the selection of patients at higher relapse risk for prospective studies. Unfortunately, there are many patients who present with metastatic disease that currently is not likely to be curable. There is no standard chemotherapy regimen for these patients; however, there is new-found hope with a currently accruing clinical trial exploring a 5-fluorouracil-based chemotherapy combination in this patient population.

Multimodality management of urachal carcinoma: the M. D. Anderson Cancer Center experience.

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.

A practical approach to bladder sampling and diagnostic reporting of pathological findings.

The most common bladder specimens are obtained from endoscopic biopsies and transurethral resections (TURB), both of which sample subepithelial tissue of varying depth. Other specimens can be obtained from cystectomy, cystoprostatectomy, pelvic exenteration ("en bloc" resection), and partial cystectomy including resection of diverticulae and surgical excision of a urachal carcinoma. The correct assessment of bladder specimens may provide clinically relevant diagnostic and prognostic data. This protocol is intended to assist pathologists in providing clinically useful information as a result of examination of surgical specimens.

Cáncer del uraco / Urachal cancer

Se presenta el caso clínico de un hombre de 75 años que consulta por baja de peso, dolor, masa infraumbilical asociado a constipación y dificultad para orinar, de 12 meses de evolución. Se estudió con ECO, TAC, enema baritado, colonoscopia y cistoscopia. Se efectuó resección en bloque de un tumor uracal con pared abdominal infraumbilical comprometida, onfalectomía, asa de íleon adherida, rectosigmoides y lecho vesical, reconstituyendo inmediatamente el tránsito con anastomosis ileoileal y colorrectal. El estudio histológico reveló un adenocarcinoma mucinoso del uraco. Se clasificó en estadio III. El paciente presenta buena evolución, sin recidiva a los 6 meses del postoperatorio, corroborada con endoscopia y TAC. El cáncer del uraco es un cuadro rarísimo, habiéndose comunicado alrededor de 450 casos en la literatura. Su tratamiento es esencialmente quirúrgico y el pronóstico a largo plazo es pobre, con una sobrevida a 5 años entre 6 a 50 por ciento

Adenocarcinoma of bladder.

Forty-eight patients with adenocarcinoma (21 urachal and 27 non-urachal) of the bladder were treated at the Tata Memorial Hospital between 1976 and 1992. The study group consisted of 32 men and 16 women. The urachal tumours were more common in younger patients (mean age: 49 years) than were non-urachal tumours (mean age: 58 years). The overall 5-year survival in this series was 37%. Stage and grade were powerful predictors of outcome. Patients with non-urachal tumours showed an overall survival rate of 29.9% compared with 45.7% in patients with urachal tumours (P= 0.14). Radical cystectomy was the most common treatment modality in patients with non-urachal tumours and yielded an overall 5-year survival of 35%. Patients with urachal tumours were treated with either partial cystectomy or radical cystectomy. The 5-year survival following partial cystectomy was 56.3% compared with 25.9% following a radical cystectomy and the difference between the two was not statistically significant (P = 0.76).