%0 Journal Article
%T Isolated Optic Nerve Glioma in Children With and Without Neurofibromatosis: Retrospective Characterization and Analysis of Outcomes
%A Amar Gajjar
%A Dima Hamideh
%A Ibrahim Qaddoumi
%A Julie H. Harreld
%A Mary Ellen Hoehn
%A Paul D. Klimo
%J Journal of Child Neurology
%@ 1708-8283
%D 2018
%R 10.1177/0883073818758737
%X Isolated optic nerve glioma is a rare tumor with no consensus for the best therapeutic approach. Therefore, tumor control and preservation of visual function remain a challenge. In this retrospective study, we describe our experience over 30 years in a single-institutional cohort of children with isolated optic nerve glioma, focusing on treatments and visual outcomes. Seventeen children were followed for a median period of 8 years (range, 2-22 years). Diagnosis was based on typical neuroradiologic findings, and 3 patients had histologic confirmation of their tumors. In our study, conservative management preserved the vision of most patients with neurofibromatosis type 1 (NF1). NF1-related optic nerve gliomas were less often treated but were associated with a lower probability of progression and with occasional spontaneous regression. Sporadic tumors more frequently exhibited aggressive clinical behavior with a higher propensity for posterior extension, often requiring surgical intervention
%K isolated optic nerve glioma
%K childhood
%K neurofibromatosis
%K sporadic
%U https://journals.sagepub.com/doi/full/10.1177/0883073818758737