The Impact of Surgical Resection and Adjuvant Therapy on Survival in Pediatric Patients with Atypical Teratoid/Rhabdoid Tumor: Systematic Review and Pooled Survival Analysis

World Neurosurg. 2022 Aug:164:216-227. doi: 10.1016/j.wneu.2022.04.073. Epub 2022 Apr 22.


Background: Atypical teratoid/rhabdoid tumor (ATRT) is a rare malignant neoplasm in the pediatric population. ATRT is characterized by rhabdoid cells combined with the loss of either the INI1 (integrase interactor 1) or BRG1 (Brahma-related gene-1) protein.

Objective: To systematically review and analyze patient and tumor characteristics, prognosis, and impact of treatment on survival in pediatric patients with ATRT confirmed by alterations in INI1 or BRG1. This systematic review is the first to include only pediatric cases of ATRT confirmed with either INI1 or BRG1 alterations.

Methods: MEDLINE was searched using the terms "atypical teratoid/rhabdoid tumor" AND "paediatric/pediatric." Cases were included if confirmed by loss of INI1 or BRG1. The extracted dataset was analyzed using descriptive statistics, log-rank test, and Kaplan-Meier survival analysis via SPSS.

Results: A total of 38 articles were included in this study. The average age at diagnosis was 3 years. The most common locations reported are the supratentorial region and cerebral hemispheres. Ninety-three patients were reported to show evidence of dissemination. The average overall survival was 29 months. A significant difference in survival was noted between the tumor location groups, particularly worse outcomes for patients with spinal ATRT (P < 0.001). Extent of resection and adjuvant therapy were significant for survival (χ2 = 10.107, P = 0.018 and χ2 = 20.38, P < 0.0001, respectively).

Conclusions: ATRT of the central nervous system in pediatric populations is a rare neoplasm associated with a poor prognosis in most patients. Future studies should be directed to find a standardized treatment protocol.

Keywords: Atypical teratoid/rhabdoid tumor; BRG1; INI1; Pediatric; Survival.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Central Nervous System Neoplasms*
  • Child
  • Humans
  • Neoplasms, Neuroepithelial*
  • Rhabdoid Tumor* / metabolism
  • Rhabdoid Tumor* / surgery
  • Survival Analysis
  • Teratoma* / metabolism
  • Teratoma* / surgery