The role of BRAF V600E mutations in pediatric low-grade glioma (PLGG) is debated. Lassaletta and colleagues evaluated two cohorts of PLGG for BRAF V600E mutations and the association with clinical outcome. Although PLGG, overall, has favorable disease control with surgery and adjuvant therapy, patients with BRAF mutations had significantly worse disease control and progression-free survival. Outcomes were even worse with BRAF mutation when there was a less than total resection or coexisting mutation in CDKN2A. With 17% of PLGGs harboring BRAF V600E mutation, the authors argue that this may represent a distinct entity with poor prognosis which may respond to additional targeted therapy.
A notable proportion of PLGGs harbor BRAF V600E mutations, which may be associated with worse disease control, even with adjuvant therapy, and could potentially benefit from targeted therapies.
