title: Angiocentric neuroepithelial tumor (ANET): a new epilepsy-related clinicopathological entity with distinctive MRI.
abstract: Several types of glioneuronal tumors are known to induce intractable partial seizures in children and adults. The most frequent are dysembryoplastic neuroepithelial tumors (DNETs) and gangliogliomas. We report here a new clinicopathological entity within the spectrum of glioneuronal tumors observed in 10 children who underwent surgery for refractory epilepsy. These tumors demonstrate a unique, pathognomonic histological pattern and a specific appearance at magnetic resonance imaging (MRI). The most striking neuropathological feature is an angiocentric polarity of the tumor with gliofibrillary acidic protein (GFAP) positive fusiform and bipolar astrocytic cells arranged around blood vessels (perivascular cuffing with tumoral astrocytes). Characteristic MRI findings include involvement of cortical gray and white matter, intrinsically high signal on T1-weighted images, as well as a stalk like extension to the ventricle. Immunohistochemical neuronal markers (neurofilament protein, synaptophysin and chromogranin) confirm the presence of a neuronal cell component. Therefore, the term angiocentric neuroepithelial tumor (ANET) is proposed.
authors.affiliation: Department of Neuropathology, Université Paris-Descartes, Faculté de Médecine, Paris, France. arielle.lellouch@nck.ap-hop-paris.fr
authors.forename: Arielle
authors.initials: A
authors.lastname: Lellouch-Tubiana
authors.forename: Nathalie
authors.initials: N
authors.lastname: Boddaert
authors.forename: Marie
authors.initials: M
authors.lastname: Bourgeois
authors.forename: Martine
authors.initials: M
authors.lastname: Fohlen
authors.forename: Anne
authors.initials: A
authors.lastname: Jouvet
authors.forename: Olivier
authors.initials: O
authors.lastname: Delalande
authors.forename: David
authors.initials: D
authors.lastname: Seidenwurm
authors.forename: Françis
authors.initials: F
authors.lastname: Brunelle
authors.forename: Christian
authors.initials: C
authors.lastname: Sainte-Rose
mesh_terms: D000293:Adolescent; D001932:Brain Neoplasms / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D004827:Epilepsy / Q000209:etiology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D018302:Neoplasms, Neuroepithelial / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology*; D011859:Radiography