Online Article: Case ReportPerivascular Epithelioid Cell Tumor The First Malignant Case Report in the PancreasMourra, Najat MD, PhD*; Lazure, Thierry MD†; Colas, Chrystelle MD‡; Arrive, Lionel MD, PhD§; de Gramont, Aimery MD, PhD∥Author Information *Departments of Pathology §Radiology ∥Oncology, Hôpital Saint-Antoine ‡Oncogenetic Laboratory, Pitié-Salpêtrière, AP-HP, Paris †Pathology Department, Hôpital Kremlin-Bicêtre, AP-HP, Kremlin-Bicêtre, France This work was accepted in an abstract form at the USCAP meeting, San Antonio, TX, February 26 to March 4, 2011. The authors declare no conflict of interest. Reprints: Najat Mourra, MD, PhD, Department of Pathology, Hôpital St-Antoine, AP-HP, 184, rue du faubourg St-Antoine, 75012 Paris, France (e-mail: [email protected]). Received September 9, 2011 Accepted September 22, 2011 Applied Immunohistochemistry & Molecular Morphology: May 2013 - Volume 21 - Issue 3 - p e1-e4 doi: 10.1097/PAI.0b013e3182392bb6 Buy Metrics Abstract 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. © 2013 Lippincott Williams & Wilkins, Inc.