PLGG as a Chronic Disease

In this OncLive Peer Exchange episode, Dr. Mohamed Abdelbaki (Washington University, St. Louis) moderates a panel with Dr. Susan Chi (Dana-Farber Cancer Institute), Dr. Murali Chintagumpala (Texas Children's, Houston), and Dr. Ashley Margol (Children's Hospital Los Angeles) on treatment decision-making in pediatric low-grade glioma (PLGG).

The first clinical question addresses how PLGG functions as a chronic disease and how that framing shapes treatment goals. Dr. Chi explains that PLGG encompasses many subtypes defined by histology and biologic drivers, with management guided by age, tumor location, and extent of resection. She notes that treating a young child with a large, unresectable tumor in the optic pathway or hypothalamic region follows a very different trajectory than treating an adolescent with a temporal or cerebellar PLGG, with side-effect profiles central to decision-making.

Dr. Abdelbaki adds that after complete resection, recurrence risk is approximately 5% or less, but follow-up remains essential. In the more common scenario—midline tumors that cannot be fully resected, representing over 50–60% of cases—the disease behaves chronically: tumors may grow, plateau, or progress, and may stop growing by early adulthood. He stresses that families and providers should not panic over small increases in tumor size, as multiple therapeutic options exist. Dr. Chi notes that even after complete resection, neurocognitive effects and seizures can persist, reinforcing the chronic nature of PLGG. Dr. Chintagumpala emphasizes that overall survival remains excellent, making prevention of treatment-related morbidities the central priority.

In the next episode, "PLGG Biology and Targeted Therapy," the panel explores how the MAPK pathway and molecular alterations such as BRAF fusions and V600E mutations shape tumor behavior, treatment selection, and the growing role of oral targeted agents.