Late presentation of bladder extrophy with fungating mass: A case report

Introduction: Exstrophy of the bladder is a rare congenital anomaly usually treated in neonatal or childhood period. Patient presenting for the first time in the adulthood with fungating mass is an extreme rarity. Case Report: A 65-year-old man presented with unreconstructed ectopia vesicae with fungating mass and (acute kidney injury (AKI). The patient underwent an incisional biopsy. Histopathology showed mucinous adenocarcinoma with signet ring cells. Positron emission tomography (PET) computed tomography (CT) showed organ-confined primary bladder malignancy. He underwent radical cystectomy with cutaneous ureterostomy with primary closure of abdominal wall defect. Histopathology showed mucinous adenocarcinoma with signet ring cells (T4aN0). Discussion: Bladder exstrophy is a rare congenital anomaly, with an estimated incidence of 1 in 50,000 live births. It is more common in males (male:female, 5:1 to 6:1). The malignant potential of the exstrophied bladder mucosa is well known. Approximately 95% are adenocarcinomas and 3%-5% are squamous cell carcinomas. Most of the malignant tumors (60%) are associated with exstrophy of bladder occurring during the fourth and fifth decades of life. Conclusion: Awareness about the malignant potential of bladder extrophy and timely correction of the deformity and monitoring perhaps improves the quality of life when a multidisciplinary approach to treatment is adopted. Efforts should be made to pick up and treat such anomalies much earlier. Keywords: Adenocarcinoma, bladder extrophy, late presentation