Primary perivascular epithelioid cell tumour (PEComa) of the prostate

Perivascular epithelioid cell tumours (PEComas) are a family of rare mesenchymal tumours arising in various anatomic locations. PEComas are defined by the presence of perivascular epithelioid cells that coexpress muscle and melanotic markers, especially HMB-45. They have unpredictable biological behaviour and are mostly benign, but some tumours can become unresectable or metastatic. Surgical resection, when possible, is the best treatment option. Radiation therapy, cytotoxic chemotherapy or immunotherapy have been reported as treatment options, either alone or in combination therapy. Prostatic PEComa is extremely rare, with only 1 malignant case reported. We report the first case of prostatic PEComa, which was benign and treated with transurethral resection.