Radiotherapy-related outcomes in pediatric patients with atypical teratoid thabdoid tumor of the central nervous system

Abstract

Objective

Atypical teratoid/rhabdoid tumor (ATRT) of the central nervous system (CNS) is a rare, highly malignant tumor of early childhood. Current protocols favor multimodality treatment, but practice guidelines differ with regard to radiotherapy. The aim of this study was to evaluate outcomes and prognostic factors in pediatric ATRT receiving multimodality treatment including postoperative radiation.

Methods

Pediatric subjects with CNS ATRT treated at our institution between 2000 and 2014 were retrospectively evaluated. Kaplan-Meier method was used to estimate progression free survival (PFS) and overall survival (OS) over time. Log-rank tests were used to examine associations of demographic and treatment factors with PFS and OS.

Results

Twenty pediatric subjects (median age 23.5 months; range 4–99 months) were eligible for analysis. All underwent surgical resection, adjuvant radiotherapy, and intensive multi-agent chemotherapy. PFS at 1 year was 63.3 % (95 % CI, 38.1–80.6 %) and 2 years was 46.1 % (95 % CI, 22.9–66.5 %). OS at both 1 and 2 years was 73.0 % (95 % CI, 46.7–87.8 %). Infratentorial disease, localized disease at diagnosis, and gross total tumor resection were identified as beneficial prognostic factors in PFS and OS. Progressive disease after radiation was identified as a poor prognostic factor in OS. Radiation modality (proton versus x-ray) did not affect disease-related outcomes.

Conclusion

Modern treatment for ATRT with intensive multimodality therapy including radiotherapy results in a significant portion of subjects with long-term disease control. Initial evidence demonstrates that focal proton therapy in very young subjects is feasible with limited toxicities. Prognosis appears to be improved in localized, completely resected disease.

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