Integrated clinical and molecular landscape of disseminated pediatric low-grade glioma.
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ABSTRACT: BackgroundPediatric-type low-grade gliomas (PLGG) are the most common central nervous system (CNS) tumor in children. Many are indolent and have excellent outcomes; however, some inexplicably spread throughout the CNS leading to increased morbidity and mortality. METHODS: To better understand this rare and difficult-to-treat entity, as well as the features associated with dissemination in CNS tumors, we assembled a large international cohort (n = 269) of patients with disseminated PLGG with detailed clinical and molecular characterization, including DNA sequencing and methylome profiling. RESULTS: We identified three subgroups of patients based on the temporal and spatial distribution of dissemination. Tumors with diffuse leptomeningeal spread without a primary tumor mass and those occurring in infants had the worst clinical outcomes. The genetics overlapped substantially with that of non-disseminated PLGG, suggesting that non-genetic mechanisms are an important contributor to dissemination. Therapeutically, targeted RAS/MAPK-pathway inhibition was more effective than conventional chemotherapy as first or second-line treatment. CONCLUSIONS: In sum, this cohort increases our clinical and biological understanding of this rare disease, provides insights for improving patient care, and directs future clinical trials and basic science research.