title: [Glial and glioneuronal tumors in adults and children: main genetic alterations and towards a histomolecular classification].
abstract: Glial and glioneuronal tumors in children and adult demonstrate distinctive clinical, neuroradiological and molecular features depending on the pathological subtype and within a same subgroup according to the age. In children, gliomas are mainly located in the infratentorial part of the brain. They are most often benign and circumscribed but infiltrative tumors with dismal prognosis are recorded within the pons (DIGP) or the thalamus. Glioblastomas are very rare in children. In contrast, gliomas in adult mainly occur in the cerebral hemispheres and the most frequent subtype is glioblastoma. Glioneuronal tumors mainly occurred in children and young adults. In addition, although pilocytic astrocytomas, pleomorphic xanthoastrocytomas and gangliogliomas are classified into different subgroups according to the WHO 2007 classification, these tumors demonstrate similar neuroradiological findings: they are cystic with contrast enhancement of a mural nodule. Major advances have been made these last five years in the discovery of some master genes that are involved in gliomagenesis and point out differences between children and adults. In adults, infiltrative gliomas can be classified into two major subgroups depending on the existence or not of IDH mutations. IDH-dependent gliomagenesis encompasses diffuse grade II and grade III (they can also show additional molecular alterations such as TP53 mutation or 1p19q codeletion) and secondary glioblastomas. IDH-independent gliomagenesis include triple negative grade II gliomas, gliomatosis cerebri (grade III) and de novo glioblastomas. Pilocytic astrocytomas, pleomorphic xanthoastrocytomas and gangliogliomas share in common BRAF alterations. However, KIAA1549-BRAF fusion characterizes pilocytic astrocytomas whereas V600E BRAF mutation is mainly recorded in pleomorphic xanthoastrocytomas and gangliogliomas.
authors.affiliation: Aix-Marseille Université, Inserm, CRO2 UMR_S 911, 13385 Marseille, France. dominique.figarella-branger@univ-amu.fr
authors.forename: Dominique
authors.initials: D
authors.lastname: Figarella-Branger
authors.forename: Céline
authors.initials: C
authors.lastname: Chappe
authors.forename: Laëtitia
authors.initials: L
authors.lastname: Padovani
authors.forename: Sandy
authors.initials: S
authors.lastname: Mercurio
authors.forename: Carole
authors.initials: C
authors.lastname: Colin
authors.forename: Fabien
authors.initials: F
authors.lastname: Forest
authors.forename: Corinne
authors.initials: C
authors.lastname: Bouvier
mesh_terms: D000328:Adult; D000367:Age Factors; D001932:Brain Neoplasms / Q000145:classification / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D002648:Child; D019175:DNA Methylation; D005910:Glioma / Q000145:classification / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D006801:Humans; D036341:Intercellular Signaling Peptides and Proteins / Q000378:metabolism; D009154:Mutation / Q000235:genetics; D014944:World Health Organization
chemical_list: D036341:Intercellular Signaling Peptides and Proteins
keywords: adult; children; gliomas; glioneuronal tumors; histomolecular classification