Optic Pathway (Opticochiasmatic) and Hypothalamic Gliomas

Abstract

Optic pathway gliomas (OPGs) are typically low-grade neoplasms arising in pediatric patients, often with a history of neurofibromatosis-1 (NF-1). OPGs typically arise during the first decade of life. The mean age at diagnosis is 4–5 years. Clinical symptoms of OPGs often include visual loss, proptosis, hydrocephalus, precocious puberty, or hypopituitarism. The prevalence of NF-1 in children with OPGs is 60 %. Conversely, nearly 20 % of patients with NF-1 develop OPGs. In rare cases, an OPG can present as an intrasellar mass.