Unique presentation of retroperitoneal malignant perivascular epithelioid cell tumor (PEComa) in a young male

Perivascular Epithelioid Cell tumors (PEComas) are rare mesenchymal tumors composed of epithelioid and spindle cells that can be found almost anywhere in the body with predominance for abdominopelvic locations. They are usually diagnosed in middle-aged females and are associated with tuberous sclerosis complex. This report describes an unusual presentation of a malignant retroperitoneal PEComa in an otherwise healthy, young male. Treatment included radical nephrectomy and adrenalectomy followed by medical oncology evaluation.