LG-74: STRATEGIES FOR THE SURGICAL MANAGEMENT OF PEDIATRIC OPTIC PATHWAY GLIOMAS - EXPERIENCE WITH 100 PATIENTS

INTRODUCTION: Pediatric optic pathway gliomas (OPGs) are often considered benign, but can have detrimental effects on the quality of life, impair vision and are a potentially lethal disease. The aim of this study is to report the characteristics and outcomes of surgically treated pediatric OPGs and to identify candidates for different treatment strategies. METHODS: Retrospective chart review of consecutive pediatric patients with surgically treated OPGs by a single surgeon at our institution from 1985-2015. Three treatment pathways were defined: (1) surgery without planned adjuvant therapy; (2) surgery with planned adjuvant therapy; and, (3) patients with prior treatment. RESULTS: 100 patients - 55 male and 45 female - were included in analysis. 8 patients had NF1. Pathology revealed pilocytic astrocytoma (45%), pilomyxoid astrocytoma (9%), ganglioglioma (3%), and unknown (35%). Radiologic location of the tumor was: hypothalamic 86%, involvement of only chiasm and/or tract in 12%. Median age at diagnosis was 4 years, median age at surgery was 6 years, and median time from diagnosis to surgery was 1 year. Pathway 1: 39 patients; median PFS 73 ± 17 months; OS rate 74%; median follow-up 117 months. Pathway 2: 10 patients; median PFS 29 ± 15 months; OS rate 70%; median follow-up 59 months. Pathway 3: 51 patients; median PFS 33 ± 8 months; OS rate 80%; median follow-up was 73 months. CONCLUSION: The role of surgery in the treatment of pediatric OPGs depends on patient characteristics and tumor biology. With the adequate therapeutic strategy, long-term PFS and OS can be achieved.