pLGG Subtypes

pLGG is a heterogenous group of solid brain tumors^1,2

Lily, lives with pLGG.
Lives for dancing.

Most of these tumors have a BRAF alteration (fusion or mutation)²

Overview of BRAF alterations in pediatric low-grade glioma (pLGG)^3-5

Diagnosis	Typical location	BRAF alteration/frequency
Pilocytic astrocytoma		KIAA1549-BRAF: 70-80% BRAF V600E: 3-5%
Ganglioglioma		KIAA1549-BRAF: 10-15% BRAF V600E: 40-50%
Desmoplastic infantile astrocytoma/ganglioglioma		KIAA1549-BRAF: 2-5% BRAF V600E/D: 40-60%
Diffuse leptomeningeal glioneuronal tumor		KIAA1549-BRAF: 75%
Dysembryoplastic neuroepithelial tumor		BRAF V600E: ~3-10%
Pleomorphic xanthoastrocytoma		BRAF V600E: 80-90%

Talk to your patients about early comprehensive BRAF testing in pLGG

Download the Discussion Guide

Not all diagnostic tests can identify all BRAF alterations³

Genomic testing in pLGG is critical to identifying potential targeted treatment strategies. Common tests can be used to detect BRAF point mutations and/or fusions, which characterize about 2/3 of pLGG cases.³

Common testing techniques used to detect BRAF alterations³

DNA and/or RNA sequencing (eg, Next-generation sequencing)

Immunohistochemistry

Fluorescent in situ hybridization

Polymerase chain reaction

Sequencing-based approaches (eg, Next-generation sequencing) enable comprehensive detection of BRAF alterations in a single test.³

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BRAF Alterations

References: 1. Hauser P. Classification and treatment of pediatric gliomas in the molecular era. Children (Basel). 2021;8(9):739. doi:10.3390/children8090739 2. Louis DN, Perry A, Wesseling P, et al. The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol. 2021;23(8):1231-1251. doi:10.1093/neuonc/noab106 3. Ryall S, Tabori U, Hawkins C. Pediatric low-grade glioma in the era of molecular diagnostics. Acta Neuropathol Commun. 2020;8(1):30. doi:100.1186/s40478-020-00902-z 4. Behling F, Schittenhelm J. Oncogenic BRAF alterations and their role in brain tumors. Cancers (Basel). 2019;11(6):794. doi:10.3390/cancers11060794 5. Andrews, LJ, Thornton ZA, Saincher. Prevalence of BRAF V600E in glioma and use of BRAF inhibitors in patients with BRAF V600E mutation-positive glioma: systemic review. Neuro Oncol. 2022; 24(4):528-540. doi:10.1093/neuonc/noab247 6. Sholl LM. A narrative review of BRAF alterations in human tumors: diagnostic and predictive implications. Precis Cancer Med. 2020; 3(26);1-15. doi:10.21037/pcm-20-39 7. Yaeger R, Corcoran RB. Targeting alterations in the RAF-MEK pathway. Cancer Discov. 2019;9(3):329-341. doi:10.1158/2159-8290.CD-18-1321 8. Srinivasa K, Cross KA, Dahiya S. BRAF alterations in central and peripheral nervous system tumors. Front Oncol. 2020;10:574974. doi:10.3389/fonc.2020.574974 9. Sun Y, Alberta JA, Pilarz C. A brain-penetrant RAF dimer antagonist for the noncanonical BRAF oncoprotein of pediatric low-grade astrocytomas. Neuro Oncol. 2017;19(6):774-785. doi:10.1093/neuonc/now261 10. Penman CL, Faulkner C, Lowis SP, Kurian KM. Current understanding of BRAF alterations in diagnosis, prognosis, and therapeutic targeting in pediatric low-grade gliomas. Front Oncol. 2015;5:54. doi:10.3389/fonc.2015.00054 11. Ryall S, Zapotocky M, Fukuoka K, et al. Integrated molecular and clinical analysis of 1,000 pediatric low-grade gliomas. Cancer Cell. 2020;37(4):569-583.e5. doi:10.1016/j.ccell.2020.03.011 12. Cohen AR. Brain tumors in children. N Engl J Med. 2020; 386(20):1922-1931. doi:10.1056/NEJMra2116344 13. Lassaletta A, Zapotocky M, Mistry M, et al. Therapeutic and prognostic implications of BRAF V600E in pediatric low-grade gliomas. J Clin Oncol. 2017;35(25):2934-2941. doi:10.1200/JCO.2016.71.8726