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Review
. 2021 Apr;32(2):181-190.
doi: 10.1016/j.nec.2020.12.001. Epub 2021 Feb 18.

Pediatric Gliomas: Molecular Landscape and Emerging Targets

Sophie M Peeters  1 , Yagmur Muftuoglu  1 , Brian Na  2 , David J Daniels  3 , Anthony C Wang  4
Affiliations
Review

Pediatric Gliomas: Molecular Landscape and Emerging Targets

Sophie M Peeters et al. Neurosurg Clin N Am. 2021 Apr.

Abstract

Next-generation sequencing of pediatric gliomas has revealed the importance of molecular genetic characterization in understanding the biology underlying these tumors and a breadth of potential therapeutic targets. Promising targeted therapies include mTOR inhibitors for subependymal giant cell astrocytomas in tuberous sclerosis, BRAF and MEK inhibitors mainly for low-grade gliomas, and MEK inhibitors for NF1-deficient BRAF:KIAA fusion tumors. Challenges in developing targeted molecular therapies include significant intratumoral and intertumoral heterogeneity, highly varied mechanisms of treatment resistance and immune escape, adequacy of tumor penetrance, and sensitivity of brain to treatment-related toxicities.

Keywords: Diffuse midline glioma; Histone mutation; Molecular genetics; Next-generation sequencing; Pediatric glioma; Targeted therapy.

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Conflict of interest statement

Disclosure The authors have no conflicts relevant to this manuscript to disclose. The authors of this article have no financial conflict of interest to disclose.