Open AccessAngiocentric Neuroepithelial Tumor (ANET): A New Epilepsy‐Related Clinicopathological Entity with Distinctive MRI
Author(s) -
LellouchTubiana Arielle,
Boddaert Nathalie,
Bourgeois Marie,
Fohlen Martine,
Jouvet Anne,
Delalande Olivier,
Seidenwurm David,
Brunelle Françis,
SainteRose Christian
Publication year - 2005
Publication title -
brain pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.986
H-Index - 132
eISSN - 1750-3639
pISSN - 1015-6305
DOI - 10.1111/j.1750-3639.2005.tb00112.x
Subject(s) - synaptophysin , pathognomonic , neuroepithelial cell , pathology , chromogranin a , ganglioglioma , magnetic resonance imaging , immunohistochemistry , epilepsy , cortical dysplasia , biology , medicine , neuroscience , radiology , genetics , disease , stem cell , neural stem cell
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.