Abstract
Perivascular epithelioid cell neoplasm (PEComa) is a rare mesenchymal-derived tumor with specific histological and immunohistochemical manifestations. Given the rarity of bladder PEComa, standardized diagnostic and therapeutic protocols have yet to be established. This article presents a case of bladder PEComa that was ultimately diagnosed after a six-year delay and reviews recent studies to improve clinicians' understanding of this tumor and minimize the risk of misdiagnosis.