REVIEW article
Front. Mol. Neurosci.
Sec. Brain Disease Mechanisms
Volume 18 - 2025 | doi: 10.3389/fnmol.2025.1662414
This article is part of the Research TopicInnovative Approaches in Glioma Therapy: Exploring New Therapeutic Frontiers- Volume IIView all articles
IDH Mutant High-Grade Gliomas
Provisionally accepted
- 1Perth Children's Hospital, Perth, Australia
- 2The University of Western Australia, Perth, Australia
- 3The Kids Research Institute Australia, Nedlands, Australia
- 4Nationwide Children's Hospital, Columbus, United States
- 5The Children's Hospital of Philadelphia, Philadelphia, United States
- 6University of Pennsylvania Perelman School of Medicine, Philadelphia, United States
Select one of your emails
You have multiple emails registered with Frontiers:
Notify me on publication
Please enter your email address:
If you already have an account, please login
You don't have a Frontiers account ? You can register here
Gliomas are the most common type of malignant primary central nervous system (CNS) tumors, resulting in significant morbidity and mortality in children and adolescent and young adult (AYA) patients. The discovery of mutations in isocitrate dehydrogenase (IDH) genes has dramatically changed the classification and understanding of gliomas. IDH mutant gliomas have distinct clinical, pathological, and molecular features including a favorable prognosis and response to therapy compared to their wildtype counterparts. Although more common in adults, 5-15% of pediatric gliomas have IDH mutations. In this review, we provide a comprehensive summary of the current knowledge on IDH mutant high-grade gliomas (HGG), including their biology, clinical features, diagnosis, treatment, and prognosis. We also discuss future directions in research and clinical management with particular attention to the AYA cohort.
Keywords: IDH mutation, high-grade glioma, IDH inhibitors, clinical trials, pediatric, Adolescent and young adults
Received: 09 Jul 2025; Accepted: 15 Aug 2025.
Copyright: © 2025 Valvi, Fouladi, Fisher and Gottardo. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Santosh Valvi, Perth Children's Hospital, Perth, Australia
Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.