LGG-18. GANGLIOGLIOMAS IN CHILDREN - MD ANDERSON CANCER CENTER EXPERIENCE. Issue 2 (22nd June 2018) - Baig, Muhammad
Abstract: INTRODUCTION: Ganglioglioma (GG) is a rare mixed glial-neuronal neoplasm accounting for 0.5-5% of all pediatric brain tumors. Optimal therapeutic strategy remains unknown. This retrospective study evaluates the prognostic factors and outcomes of this rare neoplasm. PATIENTS AND METHODS: Retrospective review of 55 patients under the age of 21 years diagnosed with GG at our institution, from 1992 to 2012 was performed. Patient demographics, clinical history, radiological features and treatment data were collected and analyzed. Kaplan-Meier survival and Cox-regression analyses were performed to assess the overall survival (OS) and progression-free survival (PFS). RESULTS: The mean age at diagnosis was 11.8 years (range 1-21 years) with a median follow-up period of 9.5 years (95% CI: 5.7–11.5). Fifty three patients had low grade GG and 2 patients had anaplastic GG. Twenty five patients had tumor progression, whose median progression free survival time was 12 years. Six patients with low grade GG progressed to a higher grade, with median survival of 9.1 month after transformation. The 5 and 10 years PFS and OS were 65%, 57%, 96% and 86% respectively. Tumor location and extent of resection were significant factors for PFS and presence of metastasis attained significance for OS. CONCLUSIONS: We present one of the largest retrospective to-date studies in pediatric GG. Notably the extent of tumor, can further stratify GG into low- and high-risk groups among children. Considering the rarity of these tumors, prospective collaborative multi-institutional studies are warranted to delineate treatment consensus and investigate prognostic factors to improve the outcome.