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Review
. 2024 Sep 5;13(17):1492.
doi: 10.3390/cells13171492.

Emerging and Biological Concepts in Pediatric High-Grade Gliomas

Abigail Yoel  1   2 , Shazia Adjumain  1   2 , Yuqing Liang  1   2 , Paul Daniel  1   2 , Ron Firestein  1   2 , Vanessa Tsui  1   2
Affiliations
Review

Emerging and Biological Concepts in Pediatric High-Grade Gliomas

Abigail Yoel et al. Cells. .

Abstract

Primary central nervous system tumors are the most frequent solid tumors in children, accounting for over 40% of all childhood brain tumor deaths, specifically high-grade gliomas. Compared with pediatric low-grade gliomas (pLGGs), pediatric high-grade gliomas (pHGGs) have an abysmal survival rate. The WHO CNS classification identifies four subtypes of pHGGs, including Grade 4 Diffuse midline glioma H3K27-altered, Grade 4 Diffuse hemispheric gliomas H3-G34-mutant, Grade 4 pediatric-type high-grade glioma H3-wildtype and IDH-wildtype, and infant-type hemispheric gliomas. In recent years, we have seen promising advancements in treatment strategies for pediatric high-grade gliomas, including immunotherapy, CAR-T cell therapy, and vaccine approaches, which are currently undergoing clinical trials. These therapies are underscored by the integration of molecular features that further stratify HGG subtypes. Herein, we will discuss the molecular features of pediatric high-grade gliomas and the evolving landscape for treating these challenging tumors.

Keywords: H3G34V/R; H3K27M; clinical trials; high-grade glioma; immunotherapy; pediatric; treatment.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Locations of grade 4 high−grade glioma subtypes in the brain with mutation frequency.
See this image and copyright information in PMC

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