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CC BY 4.0 · Indian Journal of Neurosurgery 2023; 12(02): 104-115
DOI: 10.1055/s-0043-1771192
Review Article

H3 K27M-Altered Diffuse Midline Gliomas: A Review

Karol Wiśniewski
1   Department of Neurosurgery, Royal Melbourne Hospital, Melbourne, Australia
2   Department of Neurosurgery and Neurooncology, Medical University of Łódź, Łódź, Poland
,
Andrew Ghaly
1   Department of Neurosurgery, Royal Melbourne Hospital, Melbourne, Australia
,
Kate Drummond 
1   Department of Neurosurgery, Royal Melbourne Hospital, Melbourne, Australia
3   Department of Surgery, Royal Melbourne Hospital, University of Melbourne, Melbourne, Australia
,
Andreas Fahlstrӧm 
1   Department of Neurosurgery, Royal Melbourne Hospital, Melbourne, Australia
4   Department of Medical Sciences, Section of Neurosurgery, Uppsala University, Uppsala, Sweden
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Abstract

Diffuse midline glioma H3 K27M-altered is a recently renamed high-grade glioma in the 2021 World Health Organization (WHO) Classification of Central Nervous System Tumors, previously being labelled diffuse midline glioma H3 K27M-mutant in the 2016 update and diffuse intrinsic pontine glioma prior to 2016. After identification of multiple alterations causing H3 K27 hypomethylation, the definition of this tumor subtype was changed. To further characterize this new entity in both the pediatric and adult population, we conducted a review of the current literature, investigating genetic, epidemiological, clinical, radiological, histopathological, treatment and prognostic characteristics, particularly highlighting the differences between adults and children. This tumor is more common in children, and has a poorer prognosis. Additionally, childhood H3 K27-altered gliomas are more common in the brainstem, but more common in the thalamus in adults. Sadly, limited treatment options exist for these tumors, with radiotherapy the only treatment shown to improve overall survival.

Keywords

diffuse midline glioma - H3.1 or H3.2 K27-mutant - H3-wildtype with EZHIP overexpression - EGFR-mutant - H3.3 K27-mutant


Publication History

Article published online:
26 July 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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