The impact of surgical resection and adjuvant therapy on survival in paediatric patients with Atypical Teratoid Rhabdoid Tumour: Systematic review and pooled survival analysis

Abstract

Background
Atypical teratoid/rhabdoid tumours (AT/RT) is a rare malignant neoplasm in the paediatric population. AT/RT is characterised by rhabdoid cells combined with the loss of either the INI1 or BRG1 protein.

Objective
To systematically review and analyse patient and tumour characteristics, prognosis, and impact of treatment on survival in paediatric patients with AT/RT confirmed by alterations in INI1 or BRG1. This systematic review is the first only to include paediatric cases of AT/RT confirmed with either INI1 or BRG1 alterations.

Methods
MEDLINE was searched using the terms "atypical teratoid/rhabdoid tumour" AND "paediatric/pediatric". Cases were included if confirmed by loss of INI1 or BRG1. The extracted dataset was analysed 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 three 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 tumour location groups, particularly worst outcomes for patients with spinal AT/RT (p = <0.001). Extent of resection and adjuvant therapy were significant for survival (Chi-square = 10.107, p = 0.018) and (Chi-square = 20.38, p = < 0.0001), respectively.

Conclusion
AT/RT of the central nervous system in paediatric populations is a rare neoplasm associated with a poor prognosis in most patients. Future studies should be directed to find a standardised treatment protocol.