An unusual natural history of a rare bladder tumor: Primitive neuroectodermal tumor.

A primary primitive neuroectodermal tumor (PNET) of the urinary bladder is a very rare bladder tumor. There are few described cases in the literature to date. We presented a 70-year-old man with primary nonmetastatic bladder PNET. The diagnosis was confirmed according to immunohistochemistry evaluation. The patient underwent bladder-sparing protocol using radiotherapy and standard systemic multidrug chemotherapy (vincristine + doxorubicin + cyclophosphamide). At the end of three years of follow-up, we performed radical cystoprostatectomy due to newly diagnosed high-grade transitional cell carcinoma of the bladder at the site far from the primary bladder PNET. In conclusion, multimodal bladder sparing protocol including radiotherapy and standard systemic chemotherapy can be used for the management of primary bladder PNET with acceptable oncological outcomes.