A Rare Case of an Adult with Untreated Bladder Exstrophy Presenting with Signet-Ring Cell Adenocarcinoma of Urinary Bladder

Abstract

Untreated bladder exstrophy in an adult is rare, as the defect is obvious and primary reconstruction is usually done in infancy. There are less than 90 reported cases of primary adenocarcinoma in an untreated bladder exstrophy in literature and only two such case reports from India. Of these, only one case was of signet-ring cell type of mucinous adenocarcinoma. Here we report the second case of signet-ring cell adenocarcinoma in a 63 year old male with untreated bladder exstrophy (oldest patient in literature), to highlight the extreme rarity, yet distinct possibility and challenges faced in surgical management of such cases.

Management of cavernous abdominal wall defects post radical cystectomy in adult exstrophy complex

“…Such a repair, however, may not be feasible in extremely large defects with unavailability of adequate skin locally. Savalia et al reported managing the abdominal wall defect in a similar case using rectus abdominis rotation flap based on inferior epigastric artery 4. Such repairs that use the locally available skin or require mobilisation of the rectus may lead to further weakening of abdominal wall and ileal conduit-related complications.…”