Multifocal retroperitoneal and pelvic PEComas mimicking liposarcoma: A case report and review of literature.

ABSTRACT

Perivascular epithelioid cell neoplasms (PEComa) constitute a rare, but increasingly recognized family of seemingly distinct mesenchymal tumors which can occur in any part of the body. Due to their rarity, radiological descriptions of PEComas in the current literature are few and non-specific, making diagnosis difficult, though some common imaging features have been reported. We present an unusual case of multifocal retroperitoneal and pelvic PEComas, mimicking liposarcoma, subsequently treated with open surgery.