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.
Siefker-Radtke
Arlene
A
The University of Texas MD Anderson Cancer Center, Department of Genitourinary Medical Oncology, 1155 Herman Pressler--Unit 1374, Houston, TX 77030-3721, USA. asiefker@mdanderson.org
D000230:Adenocarcinoma / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D002295:Carcinoma, Transitional Cell / Q000175:diagnosis / Q000473:pathology; D002945:Cisplatin / Q000008:administration & dosage; D002986:Clinical Trials as Topic; D017322:Clinical Trials, Phase II as Topic; D003131:Combined Modality Therapy; D015653:Cystectomy / Q000379:methods*; D003841:Deoxycytidine / Q000008:administration & dosage / Q000031:analogs & derivatives; D003937:Diagnosis, Differential; D005260:Female; D005472:Fluorouracil / Q000008:administration & dosage; D006417:Hematuria / Q000209:etiology; D006801:Humans; D002955:Leucovorin / Q000008:administration & dosage; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011446:Prospective Studies; D012307:Risk Factors; D014472:Umbilicus / Q000601:surgery; D014496:Urachal Cyst / Q000473:pathology / Q000601:surgery; D014497:Urachus / Q000473:pathology*; D001749:Urinary Bladder Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D000093542:Gemcitabine
D003841:Deoxycytidine; D002945:Cisplatin; D002955:Leucovorin; D005472:Fluorouracil; D000093542:Gemcitabine