Toggle Main Menu Toggle Search

Open Access padlockePrints

A systematic study of molecular diagnosis, treatment, and prognosis in infant-type hemispheric glioma: An individual patient data meta-analysis of 164 patients

Lookup NU author(s): Professor Simon BaileyORCiD

Downloads


Licence

This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).


Abstract

© The Author(s) 2025. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.Background: Due to the novelty and rarity of infant-type hemispheric glioma (IHG), optimal treatment and factors determining clinical outcomes are yet to be established. Methods: We curated a series of 164 patients with IHG; 155 identified by methodical literature search and nine additional patients contributed by collaborators. Results: All tumors were hemispheric, diagnosed at a median age of 3.4 (0-52) months, and frequently (95%) non-metastatic. One hundred forty-two (86.5%) tumors harbored fusions involving receptor tyrosine kinase (RTK) genes (ALK [67/142, 47%], NTRK1/2/3 [32/142, 22.5%], ROS1 [29/142, 20.4%], MET [13/142, 9.2%], and ABL2 [1/142, 0.7%]). Sixty-four percentage, 20%, and 8% of patients were treated with surgery and adjuvant chemotherapy, surgery-only, and surgery plus targeted therapy, respectively. Five patients received radiation. Three-year event-free survival (EFS) and overall survival (OS) was 49.5% [40.7-60.2] and 79.6% [72.1-87.9], respectively. Twenty-two patients succumbed to disease, of which tumor progression (8/22, 36%) and intra-cranial hemorrhage (5/22, 23%) were the most common causes. Multivariate analysis showed that the factors most associated with an increased risk of death were no treatment except for surgery and presence of residual tumor after definitive surgery. These findings present a challenging dichotomy where surgery is both a serious risk factor for early death and, when successful, a benefit. Conclusions: Together, these findings show that IHG is a fusion driven tumor of the very young that is survivable even after progression. While optimal primary therapy for patients with IHG has yet to be established, the findings of this meta-analysis suggest treatment should focus on lowering surgical morbidity and improving its success.


Publication metadata

Author(s): Chavaz L, Bagchi A, Dhanda SK, Toutain F, Pfister SM, Sturm D, Pietsch T, Gielen GH, Waha A, Clarke M, Lu C, Karremann M, Benesch M, Perwein T, Nussbaumer G, Kramm C, Massimino M, Biassoni V, Vinci M, Mastronuzzi A, van Vuurden D, Veldhuijzen van Zanten SEM, Mackay A, Jones C, Jones DTW, Guerreiro Stucklin AS, Tabori U, Hawkins C, Ryall S, Morales La Madrid A, Lassaletta A, Bailey S, Hargrave D, Chiang J, El-Ayadi M, Minniti Mancano B, Reis RM, Hagel C, Gorsi H, Silvestrini N, Gilani A, Papusha L, Klimo Jr P, Zhou X, Gajjar A, Robinson GW, von Bueren AO

Publication type: Review

Publication status: Published

Journal: Neuro-Oncology

Year: 2026

Volume: 28

Issue: 3

Pages: 776-789

Print publication date: 01/03/2026

Online publication date: 08/11/2025

Acceptance date: 29/09/2025

ISSN (print): 1522-8517

ISSN (electronic): 1523-5866

Publisher: Oxford University Press

URL: https://doi.org/10.1093/neuonc/noaf264

DOI: 10.1093/neuonc/noaf264

PubMed id: 41206756

Data Access Statement: Data available on request from the corresponding author. All data within this manuscript are available on an interactive publicly available data portal https://viz.stjude.cloud/st-jude-childrens-research-hospital/visualization/meta-analysis-infant-type-hemispheric-gliomas~2845.


Share