Perivascular epithelioid cell tumor: the first malignant case report in the pancreas.

Pancreatic perivascular epithelioid cell tumors (PEComas) are exceedingly rare neoplasms, <10 cases have been described; all were benign and occurring mostly in women. We report the first malignant pancreatic PEComa in a patient with BRCA2 mutation. A 51-year-old woman with a medical and familial history of breast adenocarcinoma, who was tested positive for BRCA2 mutation, presented with a recent history of jaundice. Imaging studies were consistent with endocrine or secondary tumor. Pathologic examination of fine needle aspiration biopsy and Whipple procedure revealed a 6-cm intrapancreatic PEComa, invading the duodenal wall with extensive necrosis and vascular invasion, leading to classify this PEComa into "malignant" category. The patient developed liver metastases 6 months after surgery. Although mostly benign, thorough sampling of the PEComa is mandatory, to rule out malignant behavior whatever the location of the tumor is.