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Journal of Child Neurology
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Pediatric Low-Grade Gliomas

Angela J. Sievert, MD, MPH

Division of Oncology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania

Michael J. Fisher, MD

Division of Oncology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, fisherm{at}email.chop.edu

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.

Key Words: low-grade • glioma • astrocytoma • pilocytic • fibrillary

Journal of Child Neurology, Vol. 24, No. 11, 1397-1408 (2009)
DOI: 10.1177/0883073809342005
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