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Optic Pathway Gliomas

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Adult CNS Radiation Oncology

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Abstract

Although comprising only 1% of all brain tumors, optic pathway glioma (OPG) is the most common optic pathway tumor. While many cases are found in young children, 20–40% are found in patients ≥15 years. Neurofibromatosis type 1 (NF1) is a well-known risk factor, with about 15–20% with NF1 developing OPG which tend to be more indolent than the non-NF1 OPG. Most are pilocytic astrocytomas, but other types of low-grade and malignant gliomas have been reported. The role of surgery is to obtain tissue diagnosis or resection in the patient with glioma confined just to the optic nerve and complete visual loss. Chemotherapy consisting of carboplatin and vincristine is a popular treatment approach to delay radiotherapy (RT) in children <10 years and preserve neurocognitive function. In NF1 patients, RT is often avoided because of the higher chance of developing vascular and neoplastic events. RT is the mainstay of treatment for non-NF1 patients who are ≥10 years. The 10-year overall survival and progression-free survival rates range from 80% to 95% and 70% to 85%, respectively, with RT. In addition, stabilization and improvement in vision have been observed in more than 70% of patients receiving RT.

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Author information

Authors and Affiliations

  1. Department of Radiation Oncology, MD Anderson Cancer Center, Houston, TX, USA

    Arnold C. Paulino

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  1. Arnold C. Paulino
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Correspondence to Arnold C. Paulino .

Editor information

Editors and Affiliations

  1. Department of Radiation Oncology, Keck School of Medicine of USC, Norris Cancer Hospital, Los Angeles, California, USA

    Eric L. Chang

  2. Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota, USA

    Paul D. Brown

  3. Department of Radiation Oncology, University of Washington Medical Center, Seattle, Washington, USA

    Simon S. Lo

  4. Department of Radiation Oncology, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada

    Arjun Sahgal

  5. Department of Radiation Oncology, Taussig Cancer Institute Cleveland Clinic, Cleveland, Ohio, USA

    John H. Suh

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Paulino, A.C. (2018). Optic Pathway Gliomas. In: Chang, E., Brown, P., Lo, S., Sahgal, A., Suh, J. (eds) Adult CNS Radiation Oncology. Springer, Cham. https://doi.org/10.1007/978-3-319-42878-9_15

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  • DOI: https://doi.org/10.1007/978-3-319-42878-9_15

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-42877-2

  • Online ISBN: 978-3-319-42878-9

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