NS-26: THE ROLE OF SURGERY IN NON-NF-ASSOCIATED PEDIATRIC OPTIC PATHWAY GLIOMAS (OPG) - IS THERE A NEW PARADIGM?

INTRODUCTION: The surgical management of pediatric OPG is variable as the indications and type of procedure are adapted individually to the tumor behavior and clinical course. This study addresses the overall role of surgery in pediatric non-NF-associated OPG. METHODS: Retrospective review of all children with diagnosed OPG who were treated and followed in a single institution between 1981 and 2015. RESULTS: 122 children with non-NF-associated OPG were identified, 69 males and 53 females. The average age at diagnosis was 9.6 years (median). The mean duration of follow up was 7.86 years. 89 children received tumor directed surgery, including 7 complete and 48 subtotal resections as well as 27 diagnostic biopsies. Initial hydrocephalus-directed surgery was performed in 38 children. 21 children were treated with surgery alone. In total, 247 surgeries were performed with an overall complication rate of 2%. The overall survival was related to tumor size and highest in children with tumors measuring more than 3 but less than 6 cm diameter (mean 302 months), smaller than 3 cm (mean 254 months) and bigger than 6 cm (mean 72 months), respectively. Best progression free survival rates were observed in children after initial treatment with surgery followed by chemotherapy or chemotherapy alone. CONCLUSION: Surgery was shown to be beneficial in terms of tumor mass reduction and avoidance of complications related to hydrocephalus. Best overall and progression free survival rates were achieved by chemotherapy adjacent to upfront surgery or chemotherapy alone depending on the initial tumor size.