Abstract

Perivascular epithelioid cell tumors (PEComa) are a very rare mesenchymal cancer with unclear malignancy potential. Primary PEComa is rare in bladder localization. We have presented a case with a primary PEComa of the bladder. The criteria for malignancy in PEComas include greater than 5 cm tumor size, high mitotic activity, necrosis, infiltration and vascular invasion. PEComas with at least 2 of these criteria are considered malignant. The diagnosis can be made only by histopathological examination because it does not show specific macroscopic features to assist in differential diagnosis with imaging methods. Surgical treatment is recommended with transurethral resection, partial cystectomy or radical cystectomy according to the size and location of the tumor in the bladder. In this case report, the diagnosis and treatment of a case with primary PEComa in bladder localization were evaluated.