• Mark V Mishra, David W Andrews, Jon Glass, James J Evans, Adam P Dicker, Xinglei Shen, and Yaacov Richard Lawrence.
• Department of Radiation Oncology, Jefferson Medical College, Philadelphia, PA, USA. Mishramv@gmail.com
• J. Neurooncol. 2012 May 1; 107 (3): 591-7.

AbstractOptic nerve gliomas (ONG) are rare astrocytic neoplasms. A paucity of literature exists on the epidemiology and outcomes of ONG. Here, we present a series of 445 cases of ONG obtained from the Surveillance, epidemiology and end results (SEER) database. Data on patient and tumor characteristics as well as initial treatment with surgery or radiation were extracted from the SEER Database. Survival rates were calculated using the Kaplan-Meier method. A multivariate analysis was performed to determine independent prognostic factors predicting mortality hazard ratios (HRs) using Cox proportional hazards modeling. The median age range at diagnosis was 5-9 years. Twenty percent of patients were over the age of 20 years. Amongst patients with information available on tumor grade (n = 131), 83% had a low-grade tumors and 17% had a high-grade tumors. Sixteen percent of patients received radiation therapy and 18.4% of patient underwent a sub- or gross total resection. The 5 year overall survival was 96% and 20% for patients with low- and high-grade tumors, respectively. In a multivariate analysis, grade was the only significant predictor of overall survival (HR 29.3, CI: 4.3, 205.4, P < 0.001). Age at diagnosis, receipt of radiation therapy, and extent of surgical resection were not significantly correlated with overall survival. In conclusion, ONG are rare tumors seen predominantly in children. The overall prognosis of high-grade tumors remains poor in all age groups despite multi-modality treatment.

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