Perivascular epithelioid cell tumor of the gastrointestinal tract

Perivascular epithelioid cell tumor (PEComa) is a rare subtype of soft tissue sarcoma that coexpresses melanocytic and smooth muscle markers. We report a case of malignant PEComa arising from the gastrointestinal tract. Definitive diagnosis of malignant PEComa is based on pathological examination and the standard treatment is complete surgical resection. Cytotoxic chemotherapy has limited efficacy in recurrent or metastatic malignant PEComa. Overactivation of the mammalian target of rapamycin (mTOR) pathway is observed in PEComa, and mTOR inhibitors have been recommended as an effective systemic treatment.