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.
机构:
Mayo Clin, Dept Pediat & Adolescent Med, Div Pediat Hematol Oncol, 200 First St SW, Rochester, MN 55905 USAMayo Clin, Dept Pediat & Adolescent Med, Div Pediat Hematol Oncol, 200 First St SW, Rochester, MN 55905 USA
Rao, Amulya A. Nageswara
Packer, Roger J.
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机构:
Childrens Natl Med Ctr, Brain Tumor Inst, Ctr Neurosci & Behav Med, Washington, DC 20010 USA
George Washington Univ, Dept Neurol & Pediat, Washington, DC USAMayo Clin, Dept Pediat & Adolescent Med, Div Pediat Hematol Oncol, 200 First St SW, Rochester, MN 55905 USA