Purpose of ReviewPediatric low-grade gliomas (pLGGs) often result in significant long-term morbidities despite high overall survival rates. This review aims to consolidate the current understanding of pLGG biology and molecular features and provide an overview of current and emerging treatment strategies.Recent FindingsSurgical resection remains a primary treatment modality, supplemented by chemotherapy and radiotherapy in specific cases. However, recent advances have elucidated the molecular underpinnings of pLGGs, revealing key genetic abnormalities such as BRAF fusions and mutations and the involvement of the RAS/MAPK and mTOR pathways. Novel targeted therapies, including MEK, BRAF and pan-RAF inhibitors, have shown promise in clinical trials, demonstrating significant efficacy and manageable toxicity.SummaryUnderstanding of pLGGs has significantly improved, leading to more personalized treatment approaches. Targeted therapies have emerged as effective alternatives, potentially reducing long-term toxicities. Future research should focus on optimizing therapy sequences, understanding long-term impacts, and ensuring global accessibility to advanced treatments.
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Univ Virginia Hlth Syst, Dept Neurol, Charlottesville, VA 22903 USA
Univ Virginia Hlth Syst, Dept Neurol Surg, Charlottesville, VA 22903 USA
Univ Virginia Hlth Syst, Dept Med, Charlottesville, VA 22903 USAUniv Virginia Hlth Syst, Dept Neurol, Charlottesville, VA 22903 USA
机构:
Harvard Univ, Sch Med, Massachusetts Gen Hosp, Dept Neurol, Boston, MA USAHarvard Univ, Sch Med, Massachusetts Gen Hosp, Dept Neurol, Boston, MA USA
Forst, Deborah A.
Nahed, Brian V.
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Harvard Univ, Sch Med, Massachusetts Gen Hosp, Dept Neurosurg, Boston, MA USAHarvard Univ, Sch Med, Massachusetts Gen Hosp, Dept Neurol, Boston, MA USA
Nahed, Brian V.
Loeffler, Jay S.
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Harvard Univ, Sch Med, Massachusetts Gen Hosp, Dept Radiat Oncol, Boston, MA USAHarvard Univ, Sch Med, Massachusetts Gen Hosp, Dept Neurol, Boston, MA USA
Loeffler, Jay S.
Batchelor, Tracy T.
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Harvard Univ, Sch Med, Massachusetts Gen Hosp, Dept Neurol, Boston, MA USA
Harvard Univ, Sch Med, Massachusetts Gen Hosp, Dept Radiat Oncol, Boston, MA USA
Harvard Univ, Sch Med, Massachusetts Gen Hosp, Div Hematol & Oncol, Boston, MA USAHarvard Univ, Sch Med, Massachusetts Gen Hosp, Dept Neurol, Boston, MA USA