Multiple bilateral perivascular epithelioid cell tumor (PEComa) of the kidneys

Perivascular Epithelial Cell (PEComa) Tumors are extremely rare. These tumors are often regarded as low grade Sarcomas and treated as such. We report a case of a 70 year old female with a history of nonspecific complaints and on routine CT scan had bilateral multiple renal masses with no other extra renal disease. The largest mass on the right was 11 cm x 7 cm x 11 cm and the left there were multiple smaller masses. She underwent a right radical nephrectomy that showed a PEComa and an adjacent renal cell carcinoma. Her contralateral kidney was followed for 3 years with no evidence of growth or metastasis.