Bladder exstrophy is a rare malformation. Ureteral diversion, such as ureterosigmoidostomy or a neorectal bladder, has been described. When the patients reach adulthood, cancer may arise in these reconstructions. Our aim was to perform a systematic review (all languages) of the published literature on neoplasia after urinary diversion and suggested management in cases of cancer. PubMed and Cochrane library were searched for relevant articles published within the last 20 years. All identified articles were reviewed for inclusion. Carcinoma occurring in the bladder and unreconstructed exstrophy were excluded. Out of 47 articles found, 12 matched our search criteria. The outcomes of 23 patients (including 2 from the authors' institution) were reported. Twenty-two patients with adenocarcinoma and 1 with carcinoid tumour were identified. Median age at urinary diversion was 3 (range 1-13) years. There were 20 ureterosigmoidostomies and 2 neorectal bladders. Cancer was diagnosed subsequently at a median of 31 (range 5-55) years after urinary diversion still in place (n = 18) or 21 years (range 1-30) after incomplete excision of ureteric stump when re-diverted (n = 5). The long-term outcomes of 15 patients were available. Ten died due to colorectal adenocarcinoma, and 5 were disease-free at 3 years. Patients with enteric diversion for bladder exstrophy, including those with subsequent reconstruction, are at risk of adenocarcinoma during adulthood. It is important to provide adequate surveillance. If lesions suggestive of carcinoma are seen, complete excision of the receptive bowel and urinary diversion are mandatory.
Keywords: Bladder exstrophy; Carcinoma; Rectal neobladder; Ureterosigmoidostomy.