Landscape Overview of Pediatric Low-Grade Gliomas (pLGGs)

pLGGs are the most common type of central nervous system (CNS) tumors in children, accounting for approximately 40% of pediatric CNS tumors. These tumors most frequently arise in the posterior fossa region of the brain, particularly within the cerebellum, and often present with symptoms like headaches and seizures. Symptoms vary depending on the tumor’s location and may include signs of increased intracranial pressure due to obstructive hydrocephalus. Unlike adult low-grade gliomas, which are often grade 2 and carry a high risk of progression to malignancy, pediatric cases are more likely to be grade 1 with minimal potential for malignant transformation. This biological difference informs a more conservative management approach in children when appropriate.

Surgical intervention in pLGG is highly dependent on the tumor’s size, growth rate, and location. In cases where the tumor remains stable and asymptomatic, especially if it’s in a sensitive or inaccessible area, surgery may be deferred. When necessary, resection can provide both symptom relief and histologic diagnosis. Biopsy is often considered even in nonprogressive tumors to obtain a molecular understanding, which increasingly informs treatment planning. In contrast to adults—where even indolent-appearing tumors are typically treated more aggressively due to their propensity for progression—children can often be safely monitored over time, reserving intervention for cases with clear clinical or radiologic progression.

Modern management of pLGG is increasingly biomarker-driven, with molecular profiling guiding the use of targeted therapies in recurrent or refractory cases. Advances in genomics have improved treatment precision, helping identify pathways such as BRAF alterations, which are commonly implicated in these tumors. A multidisciplinary care model—incorporating neurosurgery, oncology, radiology, and supportive services—is essential in optimizing outcomes for pediatric patients, ensuring each treatment decision is tailored to tumor biology, disease course, and the child’s overall health.