Optic pathway gliomas: a review

Abstract

Optic pathway gliomas account for 3-5% of all pediatric CNS tumors and represent the most common intrinsic optic nerve tumors. These tumors occur preferentially during the first decade of life and are particularly frequent in children with neurofibromatosis type 1. Although optic pathway gliomas are low-grade tumors, their behavior can be aggressive, and their management is often challenging. Their management includes observation, surgery, chemotherapy and radiation. The role of each modality is discussed as well as current and future developments in treatment, in particular targeted therapies that are currently being investigated.

Optic nerve glioma with complete intraocular extension

“…The best treatment plan for patients with ONG is still controversial 10 . The complexity of the disease requires individualized specific interventional strategies 7 .…”

Sellar and Parasellar Imaging

“…70 They account for 65% of all optic nerve tumors, and roughly one-third are associated with neurofibromatosis type 1. 71 They are, in fact, the most common CNS neoplasms in this syndrome, in which they have been reported in 7% to 20% of children.…”

“…71 They are, in fact, the most common CNS neoplasms in this syndrome, in which they have been reported in 7% to 20% of children. 70 Syndromic optic nerve gliomas almost always develop before age 4 and are low grade and generally indolent. 72 While it appears that sporadic gliomas are associated with worse outcomes, their presentation differs from syndromic tumors in that the latter are usually found in asymptomatic patients as part of routine screening and their natural history has not been conclusively demonstrated.…”

“…74 Calcifications are uncommon and hemorrhage is exceedingly rare. 70,72,75 Cysts may be present and are more common in sporadic tumors (Figure 12). 72 Increased T2 signal is generally not useful to evaluate involvement of the optic tracts, as it may be also related to edema.…”

Corticosteroids and Anti-Complement Therapy in Retinal Diseases

“…Optic nerve sheath meningiomas (ONSM) and optic gliomas are the two primary tumors of the optic nerve (Fried et al 2013;Glass et al 2014;Liu et al 2013;Shapey et al 2011;Shofty et al 2014;Traber et al 2015). ONSM typically present in the fourth-fifth decades of life and are more common in females; when they occur in children, they typically occur in the first decade of life and are most often associated with neurofibromatosis type 2 (Grob et al 2015;Shapey et al 2013).…”