title: Perivascular epithelioid cell tumor: the first malignant case report in the pancreas.
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.
authors.affiliation: Department of Pathology, Hôpital Saint-Antoine, 75012 Paris, France. najat.mourra@sat.aphp.fr
authors.forename: Najat
authors.initials: N
authors.lastname: Mourra
authors.forename: Thierry
authors.initials: T
authors.lastname: Lazure
authors.forename: Chrystelle
authors.initials: C
authors.lastname: Colas
authors.forename: Lionel
authors.initials: L
authors.lastname: Arrive
authors.forename: Aimery
authors.initials: A
authors.lastname: de Gramont
mesh_terms: D024682:BRCA2 Protein / Q000235:genetics; D044963:Biopsy, Fine-Needle; D004379:Duodenal Neoplasms / Q000235:genetics / Q000556:secondary*; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000235:genetics / Q000556:secondary*; D008875:Middle Aged; D009154:Mutation; D010190:Pancreatic Neoplasms / Q000235:genetics / Q000473:pathology*; D054973:Perivascular Epithelioid Cell Neoplasms / Q000235:genetics / Q000473:pathology*
chemical_list: D024682:BRCA2 Protein; C551750:BRCA2 protein, human