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 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.
Division of Oncology, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Angela J
AJ
Sievert
Michael J
MJ
Fisher
D001932:Brain Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D002648:Child; D005910:Glioma / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D006801:Humans; D008959:Models, Neurological; D009367:Neoplasm Staging