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01-10-2016 | Special Annual Issue

An integrative molecular and genomic analysis of pediatric hemispheric low-grade gliomas: an update

Authors: Alvaro Lassaletta, Michal Zapotocky, Eric Bouffet, Cynthia Hawkins, Uri Tabori

Published in: Child's Nervous System | Issue 10/2016

Abstract

Hemispheric low-grade gliomas account for the second most common location in pediatric low-grade gliomas (PLGGs) after the cerebellum. The pathological spectrum includes gangliogliomas, dysembryoplastic neuroepithelial tumors (DNETs), diffuse astrocytomas, pilocytic astrocytomas, and pleomorphic xanthoastrocytomas (PXAs), among others. Clinically, hemispheric PLGGs represent a well-recognized cause of intractable epilepsy in children and adolescents. With an excellent long-term outcome, surgery remains the cornerstone and patients with gross total resection typically do not need any further therapies. The recent literature about hemispheric PLGGs was reviewed to provide an up-to-date overview of the molecular and cell biology of these tumors. Hemispheric PLGGs can harbor multiple alterations involving BRAFV600E, FGFR, NTRK, MYB/MYBL1, IDH, and BRAF-KIAA1549 fusions. However, the clinical significance of most of these alterations is still to be defined. The role of RAS/MAPK mutations and other alterations in hemispheric PLGGs is of interest from diagnostic, prognostic, and therapeutic perspectives. Molecular testing for these tumors should be encouraged, since the findings can have an important impact not only in prognosis but also in therapeutic strategies.

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Title: An integrative molecular and genomic analysis of pediatric hemispheric low-grade gliomas: an update
Authors: Alvaro Lassaletta
Michal Zapotocky
Eric Bouffet
Cynthia Hawkins
Uri Tabori
Publication date: 01-10-2016
Publisher: Springer Berlin Heidelberg
Published in: Child's Nervous System / Issue 10/2016
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-016-3163-6

At a glance: The STEP trials

Springer Medicine

An integrative molecular and genomic analysis of pediatric hemispheric low-grade gliomas: an update

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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