Angiocentric Neuroepithelial Tumor (ANET): A New Epilepsy‐Related Clinicopathological Entity with Distinctive MRI

Brain Pathology - Tập 15 Số 4 - Trang 281-286 - 2005
Arielle Lellouch‐Tubiana1,2, Nathalie Boddaert1,3, Marie Bourgeois1,4, Martine Fohlen5, Anne Jouvet6, Olivier Delalande5, David Seidenwurm7, Françis Brunelle1,3, Christian Sainte‐Rose1,4
1Assistance Publique-Hôpitaux de Paris, Hôpital Necker-Enfants Malades, Paris, France.
2Department of Neuropathology, Université Paris-Descartes, Faculté de Médecine
3Pediatric Radiology, Université Paris-Descartes, Faculté de Médecine
4Pediatric Neurosurgery, Université Paris-Descartes, Faculté de Médecine
5Department of Pediatric Neurosurgery, Fondation Rothschild, Paris, France
6Department of Neuropathology, Hôpital Neurologique et neurochirurgical, Lyon, France.
7Radiological Associates of Sacramento, Sutter Medical Center, Sacramento, Calif.

Tóm tắt

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.

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