[A case of renal perivascular epithelioid cell tumor (PEComa)]

A 44-year-old woman was admitted with acute hepatic dysfunction. Screening computed tomography showed an enhancing mass of about 9 cm in the right renal upper pole. An open right nephrectomy was performed for the pre-operative diagnosis of renal cell carcinoma. Pathological examination revealed a perivascular epithelioid cell tumor (PEComa). PEComa is a rare tumor, which includes angiomyolipoma and lymphangiomyomatosis. It is sometimes associated with aggressive clinical behavior such as local recurrence and metastasis. She had no evidence of recurrence at 34 months follow up.