1314. Management of CNS atypical teratoid/rhabdoid tumors in pediatric age group: CCHE experience

Authors: Mohamed Reda, MBBCh, MSc, IFAANS; Mohamed Elbeltagy; Hala Taha; Nada Elkhateeb (Giza, Egypt, Arab Rep.)

Introduction: Atypical Teratoid Rhabdoid tumors (ATRTs) of the CNS are extremely rare, aggressive malignant tumors that mainly arise in pediatric age group. It account for 1 -2 % of the pediatric brain tumors. Material and methods In the period between 2009 to 2016, 21 patients with confirmed pathology ATRTs at the Children's Cancer Hospital Egypt were retrospectively reviewed and analyzed regarding age at presentation, location, single or multiple, decisions and extents of surgical resection, protocol of adjuvant therapy, clinical outcome, relapse rate and overall survival. Trying to reach predictors favor good outcome and factors predict bad prognosis Results 21 patients were diagnosed with CNS ATRTs; the median age was 1.79 year. 1 year overall survival for the 21 cases were 40.2% while 2 years overall survival were 25.1% with a median follow up period of 6.62 Range from 1.3 to 35.2 months. The extent of surgical resection doesn't differ the overall survival with the statistic P value was equal 0.984 between gross total and subtotal excision. The overall survival in cases received radiation was 70.1% while in the group didn't receive radiation was 16% with significant P Value equal 0.002. Conclusion ATRT is a very aggressive tumor with dismal prognosis. Total surgical resection of the tumor didn’t improve overall or event free survival. The only factor which improves the overall survival in our study was the radiotherapy.