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Published in: Current Neurology and Neuroscience Reports 2/2017

01-02-2017 | Neuro-Oncology (LE Abrey, Section Editor)

New Molecular Considerations for Glioma: IDH, ATRX, BRAF, TERT, H3 K27M

Authors: Michael Karsy, Jian Guan, Adam L. Cohen, Randy L. Jensen, Howard Colman

Published in: Current Neurology and Neuroscience Reports | Issue 2/2017

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Abstract

Purpose of Review

This review will discuss the role of several key players in glioma classification and biology, namely isocitrate dehydrogenase 1 and 2 (IDH1/2), alpha thalassemia/mental retardation syndrome X-linked (ATRX), B-Raf (BRAF), telomerase reverse transcriptase (TERT), and H3K27M.

Recent Findings

IDH1/2 mutation delineates oligoden-droglioma, astrocytoma, and secondary glioblastoma (GBM) from primary GBM and lower-grade gliomas with biology similar to GBM. Additional mutations including TERT, 1p/19q, and ATRX further guide glioma classification and diagnosis, as well as pointing directions toward individualized treatments for these distinct molecular subtypes. ATRX and TERT mutations suggest the importance of telomere maintenance in gliomagenesis. BRAF alterations are key in certain low-grade gliomas and pediatric gliomas but rarely in high-grade gliomas in adults. Histone mutations (e.g., H3K27M) and their effect on chromatin modulation are novel mechanisms of cancer generation and uniquely seen in midline gliomas in children and young adults.

Summary

Over the past decade, a remarkable accumulation of knowledge from the genomic study of gliomas has led to reclassification of tumors, new understanding of oncogenic mechanisms, and novel treatment strategies.
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Metadata
Title
New Molecular Considerations for Glioma: IDH, ATRX, BRAF, TERT, H3 K27M
Authors
Michael Karsy
Jian Guan
Adam L. Cohen
Randy L. Jensen
Howard Colman
Publication date
01-02-2017
Publisher
Springer US
Published in
Current Neurology and Neuroscience Reports / Issue 2/2017
Print ISSN: 1528-4042
Electronic ISSN: 1534-6293
DOI
https://doi.org/10.1007/s11910-017-0722-5

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