Pediatric Low-Grade Gliomas

Pediatric low-grade gliomas encompass a heterogeneous set of tumors of different histologies Cerebellar pilocytic astrocytomas occur most frequently followed by supratentorial diffuse fibrillary astrocytomas. Recent research has implicated activation of the RAS/RAF/MEK pathway in tumorigenesis of these tumors. Surgery is the mainstay of therapy Overall survival rates for patients whose tumors are completely resected are 90% or greater, 10 years from diagnosis, Conversely, most optic pathway/hypothalamic, deep midline, and brain stem gliomas have minimal potential for resection; these tumors can be difficult to treat and deserve special attention Combination chemotherapy is currently recommended mended as front-line adjuvant treatment for progressive or recurrent tumors. Second-line radiotherapy can also improve overall survival but is associated with more frequent and significant neurocognitive, endocrine, and other long-term toxicities.