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Review
. 2019 Sep;30(5):356-363.
doi: 10.1097/ICU.0000000000000587.

Current treatment of optic nerve gliomas

Marybeth K Farazdaghi  1   2 , William R Katowitz  1   2 , Robert A Avery  1   2   3
Affiliations
Review

Current treatment of optic nerve gliomas

Marybeth K Farazdaghi et al. Curr Opin Ophthalmol. 2019 Sep.

Abstract

Purpose of review: Optic pathway gliomas are low-grade neoplasms that affect the precortical visual pathway of children and adolescents. They can affect the optic nerve, optic chiasm, optic tracts and radiations and can either be sporadic or associated with neurofibromatosis type one. Gliomas isolated to the optic nerve (ONG) represent a subgroup of optic pathway gliomas, and their treatment remains controversial. New developments in ONG treatment have emerged in recent years, and it is necessary for clinicians to have a current understanding of available therapies.

Recent findings: The current review of the literature covers the background of and recent developments in ONG treatment, with a focus on standard chemotherapy, new molecularly targeted therapies, radiation therapy and surgical resection and debulking.

Summary: Although standard chemotherapy remains the mainstay of ONG treatment, newer molecularly targeted therapies such as mitogen-activated protein kinase kinase inhibitors and bevacizumab represent a promising new treatment modality, and clinical studies are ongoing.

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Conflict of interest statement

Conflicts of interest

R.A.A. receives support from Ozmosis Research Inc (Toronto, Canada; funding supplemented by Hoffman-LaRoche and The Hospital for Sick Children, Toronto, Canada) for his role as Lead Ophthalmology Investigator for the study “A phase II, open-labeled, multicenter, randomized controlled trial of vinblastine ± bevacizumab for the treatment of chemotherapy naïve children with unresectable or progressive low grade glioma.”

Figures

FIGURE 1.
FIGURE 1.
T2-weighted axial MRIs of a sporadic left optic nerve glioma (left image) and bilateral optic nerve gliomas in a child with neurofibromatosis type 1 (right image).
FIGURE 2.
FIGURE 2.
Fourteen-year-old male diagnosed with an isolated right optic nerve glioma at age 5 that progressed to no light perception along with progressive proptosis (top figures). Twenty months after debulking using the lateral orbitotomy approach, his proptosis and facial appearance has improved (bottom figures) with a significant reduction in relative axial proptosis.
FIGURE 3.
FIGURE 3.
Lateral orbitotomy approach and identification of the optic nerve glioma.
See this image and copyright information in PMC

References

    1. Avery RA, Fisher MJ, Liu GT. Optic pathway gliomas. J Neuroophthalmol 2011; 31:269–278. - PubMed
    1. Beres SJ, Avery RA. Optic pathway gliomas secondary to neurofibromatosis type 1. Semin Pediatr Neurol 2017; 24:92–99. - PubMed
    2. The article reviews the genetic and molecular mechanisms underlying neurofibromatosis type one (NF1)-associated optic pathway gliomas (OPGs). Recommendations for utilization of new imaging techniques and clinical management of NF1-associated OPGs are made and could alter current clinical practice.
    1. Beres SJ, Avery RA. Optic pathway gliomas. J Pediatr Neurol 2017; 15:15–24. - PubMed
    2. The article reivews the pathology and pathophysiology of sporadic OPGs. The authors discuss developments in evaluation and management of sporadic OPGs that could change clinical practice.
    1. Czyzyk E, Jozwiak S, Roszkowski M, Schwartz RA. Optic pathway gliomas in children with and without neurofibromatosis 1. J Child Neurol 2003; 18:471–478. - PubMed
    1. Dolecek TA, Propp JM, Stroup NE, Kruchko C. CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2005–2009. Neuro Oncol 2012; 14(Suppl 5):v1–v49. - PMC - PubMed

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