Oncologic Concerns in An Exstrophied Urinary Bladder - An Indian Scenario

Abstract

Exstrophy of the urinary bladder is a rare congenital anomaly which if untreated causes bladder carcinoma and intestinal tumours noted if urinary diversion is performed. It is seen that 50% of all persons afflicted with exstrophy are dead by their tenth year and 66-67% are dead by their twentieth year. It is thus a great rarity to see a case of ectopia vesicae in adulthood. Still more uncommon is to see a case of exstrophy complicated by carcinoma. Here, we report a case of papillary adenocarcinoma of ectopic urinary bladder in a 42-year-old male patient. In view of locally advanced disease, patient was given neoadjuvant chemotherapy. The case is being reported on account of its rarity to sensitise clinicians about rising incidence of carcinoma if mismanaged due to lack of protocol in oncological screening.

A Rare Case of Urinary Bladder Exstrophy Presenting With Signet Ring Cell Adenocarcinoma in an Adult

“…A thorough literature search turned up fewer than 90 occurrences of primary bladder cancer in persons with untreated exstrophy. There are just two of these occurrences that are known to have their roots in India [ 3 , 4 ].…”

“…Only about 90 primary cancer cases of untreated bladder exstrophy have been reported in the literature. Only two of them were signet ring cell mucinous adenocarcinomas [ 3 , 4 ].…”

Late presentation of ectopia vesica with malignant transformation. A case report and review

“…The deformity is usually treated in the neonatal period. Although some authors reported that 66–67% of unreconstructed ectopia vesica are dead by their third decade [ 3 ], and others documented almost normal life expectancy with reconstructed bladder exstrophy and follow up [ 4 ], nevertheless it is unusual to come across a case of unconstructed Ectopia Vesicae in late adulthood. Lack of awareness, ignorance, social embarrassment or even lack of appropriate facilities might be implicated in such delayed presentation.…”