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.
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.
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.
aDivision of Ophthalmology, The Children's Hospital of Philadelphia
bDepartment of Ophthalmology
cDepartment of Neurology, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA
Correspondence to Robert A. Avery, DO, MSCE, Division of Ophthalmology, The Children's Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104, USA. Tel: +1 215 590 2791; fax: +1 267 426 5015; e-mail: email@example.com