• World Neurosurg · Sep 2019

    Factors Influencing Survival in Children with Atypical Teratoid/Rhabdoid Tumors (AT/RT): Single institute experience in developing country.

    • S J Balaparameswara Rao, Subhas K Konar, Dhaval Shukla, Dhananjaya I Bhat, Manish Beniwal, Rao K V L Narasinga KVLN Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, India., B N Nandeesh, and B Indira Devi.
    • Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, India.
    • World Neurosurg. 2019 Sep 1; 129: e264-e272.

    BackgroundWe evaluated the factors influencing overall survival (OS) and event-free survival (EFS) in children with atypical teratoid/rhabdoid tumors (ATRTs).MethodsWe performed a retrospective study of children aged <16 years and tumor histological diagnosis of ATRT. Univariate and multivariate analyses were performed to determine the effect of individual and interdependent variables and survival.ResultsA total of 34 children had undergone surgery, with a male/female ratio of 1.8:1. On univariate analysis, the factors with statistically significant influence on OS and EFS were the extent of resection (P = 0.012 and P = 0.015, respectively), adjuvant therapy (P ≤ 0.001 and P = 0.001, respectively), and rhabdoid cell percentage (P = 0.004 and P = 0.005, respectively). On survival analysis, the median OS and EFS were better for those who had completed adjuvant therapy versus those who had not received adjuvant therapy (OS, 22.7 months vs. 3.5 months; EFS, 10.9 months vs. 3.5 months), those who had undergone gross total resection versus those who had undergone partial decompression (OS, 10.9 months vs. 2 months; EFS, 8.9 months vs. 2.5 months), and those with <50% rhabdoid cells in the biopsy specimen versus those with >50% rhabdoid cells (OS, 10.9 months vs. 3.7 months; EFS, 8.9 months vs. 3.5 months). On multivariate analysis, only the extent of resection and adjuvant therapy status had a significant influence on OS and EFS.ConclusionAchieving gross total resection should be the aim of surgery, depending on the tumor location, and these children should undergo upfront adjuvant treatment.Copyright © 2019. Published by Elsevier Inc.

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