Non-urothelial Bladder Cancer (BC) and variants of urothelial carcinoma account for up to 25 % of all BCs. Given their heterogeneity, these entities are not well represented in clinical trials and treatment remains challenging. Checkpoint inhibitor therapy has shown a role in the treatment of urothelial BC. By contrast, robust evidence regarding its use in other histological types is lacking. We aimed to provide a comprehensive update of non-urothelial and variant urothelial BC, exploring the evidence for immune checkpoint inhibitor therapy. A detailed analysis of the literature was conducted regarding epidemiology, aetiology, diagnosis, prognosis, treatment and outcomes of these patients in the immunotherapy era. A growing body of evidence suggests that immune checkpoint inhibition might have a role to play in non-urothelial BC, similarly to what happened with urothelial carcinomas.
Keywords: Bladder adenocarcinoma; Checkpoint inhibitors; Immunotherapy; Non-urothelial bladder cancer; Squamous cell bladder cancer; Variant urothelial cancer.
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