title: [Pancreatic perivascular epithelioid cell tumor (PEComa)].
abstract: Neoplasms with perivascular épithelioid-cell differentiation (PEComas) are rare tumors with a distinctive immunoreactivity for melanocytic markers. They have been described in various organs. We report an intrapancreatic PEComa discovered in a 46-year-old woman during a workup for diarrhea. CT scan showed a 1.7cm nodule in the body of the pancreas with slight-contrast enhancement at arterial time and isodense at portal time. The aspect was suggestive of an endocrine tumor despite negative somatostatin-receptor scintigraphy. Enucleation was performed. Pathologic evaluation showed a well-circumscribed intrapancreatic tumor consisting of a population of clear to eosinophilic spindle cells and a less abundant population of epithelioid cells arranged around blood vessels. Tumor cells expressed vimentin, HMB45 and actin and only focally S-100 protein, KL1, CD117 and CD34. These features were consistent with a PEComa. Pancreatic PEComas are rare, but should be included in the differential diagnostic of pancreatic clear cells tumors or pancreatic spindle- and epithelioid-cells tumors.
authors.affiliation: Service d'anatomie pathologique, hôpital Beaujon, AP-HP, université Paris-7 Denis-Diderot, 100, boulevard du Général-Leclerc, 92110 Clichy, France.
authors.forename: Martine
authors.initials: M
authors.lastname: Périgny
authors.forename: Olivier
authors.initials: O
authors.lastname: Larochelle
authors.forename: Pascal
authors.initials: P
authors.lastname: Hammel
authors.forename: Alain
authors.initials: A
authors.lastname: Sauvanet
authors.forename: Safi
authors.initials: S
authors.lastname: Dokmak
authors.forename: Jacques
authors.initials: J
authors.lastname: Belghiti
authors.forename: Philippe
authors.initials: P
authors.lastname: Ruszniewski
authors.forename: Valérie
authors.initials: V
authors.lastname: Vilgrain
authors.forename: Pierre
authors.initials: P
authors.lastname: Bedossa
authors.forename: Anne
authors.initials: A
authors.lastname: Couvelard
mesh_terms: D015622:Epithelioid Cells / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008579:Meningioma / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery*; D011859:Radiography