Treatment of pediatric low-grade gliomas Review


Authors: Sait, S. F.; Giantini-Larsen, A. M.; Tringale, K. R.; Souweidane, M. M.; Karajannis, M. A.
Review Title: Treatment of pediatric low-grade gliomas
Abstract: Purpose of Review: Pediatric low-grade gliomas and glioneuronal tumors (pLGG) account for approximately 30% of pediatric CNS neoplasms, encompassing a heterogeneous group of tumors of primarily glial or mixed neuronal-glial histology. This article reviews the treatment of pLGG with emphasis on an individualized approach incorporating multidisciplinary input from surgery, radiation oncology, neuroradiology, neuropathology, and pediatric oncology to carefully weigh the risks and benefits of specific interventions against tumor-related morbidity. Complete surgical resection can be curative for cerebellar and hemispheric lesions, while use of radiotherapy is restricted to older patients or those refractory to medical therapy. Chemotherapy remains the preferred first-line therapy for adjuvant treatment of the majority of recurrent or progressive pLGG. Recent Findings: Technologic advances offer the potential to limit volume of normal brain exposed to low doses of radiation when treating pLGG with either conformal photon or proton RT. Recent neurosurgical techniques such as laser interstitial thermal therapy offer a “dual” diagnostic and therapeutic treatment modality for pLGG in specific surgically inaccessible anatomical locations. The emergence of novel molecular diagnostic tools has enabled scientific discoveries elucidating driver alterations in mitogen-activated protein kinase (MAPK) pathway components and enhanced our understanding of the natural history (oncogenic senescence). Molecular characterization strongly supplements the clinical risk stratification (age, extent of resection, histological grade) to improve diagnostic precision and accuracy, prognostication, and can lead to the identification of patients who stand to benefit from precision medicine treatment approaches. Summary: The success of molecular targeted therapy (BRAF inhibitors and/or MEK inhibitors) in the recurrent setting has led to a gradual and yet significant paradigm shift in the treatment of pLGG. Ongoing randomized trials comparing targeted therapy to standard of care chemotherapy are anticipated to further inform the approach to upfront management of pLGG patients. © 2023, The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
Keywords: child; chemotherapy; brain tumor; glioma; neurosurgery; brain neoplasms; metabolism; radiotherapy; brain; mek inhibitors; molecularly targeted therapy; braf inhibitors; molecular targeted therapy; humans; human; glioneuronal tumors; pediatric low-grade gliomas
Journal Title: Current Neurology and Neuroscience Reports
Volume: 23
Issue: 4
ISSN: 1528-4042
Publisher: Springer  
Date Published: 2023-04-01
Start Page: 185
End Page: 199
Language: English
DOI: 10.1007/s11910-023-01257-3
PUBMED: 36881254
PROVIDER: scopus
PMCID: PMC10121885
DOI/URL:
Notes: Review -- MSK Cancer Center Support Grant (P30 CA008748) acknowledged in PubMed and PDF -- MSK corresponding authors are Sameer Farouk Sait and Matthias A. Karajannis -- Export Date: 31 May 2023 -- Source: Scopus
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  1. Kathryn Ries Tringale
    101 Tringale