Factors Influencing Survival of Children with Atypical Teratoid/Rhabdoid Tumors: A Single-Institute Experience in a Developing Country.

Abstract

We evaluated the factors influencing overall survival (OS) and event-free survival (EFS) in children with atypical teratoid/rhabdoid tumors (ATRTs). We 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. A 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. Achieving gross total resection should be the aim of surgery, depending on the tumor location, and these children should undergo upfront adjuvant treatment.