LG-16RADIOGRAPHIC AND OPHTHALMOLOGIC CHARACTERISTICS OF OPTIC PATHWAY GLIOMA IN CHILDREN WITH NEUROFIBROMATOSIS 1

Abstract

LG-16. RADIOGRAPHIC AND OPHTHALMOLOGIC CHARACTERISTICS OF OPTIC PATHWAY GLIOMA IN CHILDREN WITH NEUROFIBROMATOSIS 1 Elizabeth Unterbrink1, Benita Tamrazi1,2, Mark Borchert1,2, Ashley Margol1,2, Linda Randolph1,2, Tena Rosser1,2, Girish Dhall1,2, and Nathan Robison1,2; Children’s Hospital Los Angeles, Los Angeles, CA, USA; University of Southern California Keck School of Medicine, Los Angeles, CA, USA OBJECTIVE: To describe the natural history of optic pathway glioma (OPG) in children with neurofibromatosis 1 (NF1). METHODS: The Children’s Hospital Los Angeles (CHLA) institutional review board approved this retrospective study. Using a clinical database, we identified children with NF1 and OPG followed at CHLAwith at least 1 MRI obtained at ,12 years of age from 6/1/1997-5/31/2014. Demographic, treatment, and visual field/ acuity data were extracted. MRIs were reviewed by a neuroradiologist. RESULTS: Of 63 children with NF1 and OPG, 50 had available imaging and were included. Mean age at initial MRI was 4.8 years; mean follow-up was 6.8 years. OPG was apparent on brain MRI in 4/7 children at ,2 years (57%; 95% confidence interval[95%CI] 20-94); 19/23 by 4 years (83%; 95%CI 67-98); 29/31 by 6 years (94%; 95%CI 85-100); and 50/50 by 12 years. Among 13 children (26%) who subsequently received tumor-directed treatment, OPG was MRI-apparent in 5/6 children by 4 years (83%; 95%CI 54-100), 8/9 by 6 years (89%; 95%CI 68-100) and 13/13 by 8 years. Among children with detailed visual outcome data, visual impairment was seen in 6/20 at , 4 years (30%; 95%CI 10-50); and 26/45 by 12 years (58%; 95%CI 43-72). A subset had normalization of vision, with persistent visual abnormalities in only 14/41 children on follow-up (34%; 95%CI 20-49). DISCUSSION: A minority of children without MRI-apparent OPG at initial evaluation eventually developed symptomatic disease. The high incidence of early-onset visual abnormalities highlights the importance of early NF1 diagnosis and ophthalmologic screening. Neuro-Oncology 18:iii78–iii96, 2016. doi:10.1093/neuonc/now075.16 #The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.