Medical Student Washington University School of Medicine Saint Louis, MO, US
Introduction: Atypical teratoid rhabdoid tumors (ATRTs) are traditionally aggressive pediatric tumors. Paucity of adult cases requires thorough clinical characterization.
Methods: Comprehensive literature review of adult (age ≥ 18 years) ATRT cases was performed. Relationships between demographics and treatment modality on overall survival (OS) was assessed via Spearman’s r and Mann-Whitney’s test.
Results: In total, 102 cases were included (including one institutional case), distributed across 72 female and 30 male patients. Mean age at diagnosis was 38.6 ± 15.5 years, with a significant negative correlation between diagnostic age and OS (-0.2705, CI -0.4602-0.05713, p = 0.0113). Adjuvant radiation and chemotherapy independently conferred survival benefits (radiation: 19.0 vs. 1.25 months, p< 0.0001; chemotherapy: 22.5 vs. 3.75 months, p< 0.0001) agnostic to modality. Extent of resection did not affect overall survival (p=0.7610).
Conclusion : Adjuvant chemoradiation and younger age are associated with greater OS in adult ATRT irrespective of regimen.
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