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 t...

Full description

Saved in:
Bibliographic Details
Published in:Journal of child neurology 2009-11, Vol.24 (11), p.1397-1408
Main Authors: Sievert, Angela J., Fisher, Michael J.
Format: Article
Language:English
Subjects:
Brain Neoplasms - diagnosis
Brain Neoplasms - epidemiology
Brain Neoplasms - therapy
Child
Glioma - diagnosis
Glioma - epidemiology
Glioma - therapy
Humans
Models, Neurological
Neoplasm Staging
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:0883-0738
1708-8283
DOI:10.1177/0883073809342005