Abstract
INTRODUCTION: Bladder exstrophy is a very rare congenital anomaly and is associated with an increased incidence of bladder cancer. Most patients undergo reconstructive operations during childhood. PRESENTATION OF CASE: A 53 year-old woman was diagnosed with enteric type mucinous adenocarcinoma of the unreconstructed exstrophic bladder. Preoperative examination revealed no primary site in the gastrointestinal system. The patient underwent an anterior pelvic exenteration with bilateral ureterostomy and repairing of the anterior abdominal wall defect with a mesh. DISCUSSION: Carcinoma of the exstrophic bladder is a very rare entity. An overall number of 118 cases of cancer in unreconstructed bladder have been reported in the literature since 1895 with adenocarcinomas consisting of 90-91% and squamous cell carcinomas 8.5%. Mechanical irritation on the bladder mucosa, embryologic origin and urine carcinogens are some o the theories of cancerous metaplasia. CONCLUSION: This is the first case of bladder exstrophy cancer reported that was submitted to anterior pelvic exenteration. We propose this surgical procedure as an appropriate alternative in postmenopausal women and cancer of the unreconstructed bladder exstrophy.