Adjuvant Chemoradiation Confers a Survival Benefit in Sellar/Suprasellar Adult Atypical Teratoid Rhabdoid Tumor

Introduction: Atypical teratoid rhabdoid tumors (ATRTs), aggressive WHO grade 4 tumors typically presenting in the pediatric population and associated with a very poor prognosis, currently have a median overall survival of 11 months. Despite their usual adult presentation as sellar/suprasellar masses, no studies assessing factors associated with increased survival have focused on this entity.

Methods: A comprehensive literature review of documented cases of sellar/suprasellar ATRT in adult (age ≥ 18 years) was performed and information on patient treatment and overall survival was obtained. Spearman’s r was used to assess the relationship between age and overall survival (OS) and Mann–Whitney’s test was used to assess the impact of postsurgical treatment modalities on patient outcomes.

Results: A total of 48 patients were included, of which 45 were females and 3 were males. Mean age at diagnosis was 46.5 ± 13.8 years. Spearman’s r showed a significant negative correlation between age at diagnosis and OS (−0.3448, CI: −0.5988 to −0.02773, p = 0.0294). Exactly 35 patients had detailed information regarding postoperative treatment, those who did not undergo adjuvant chemoradiation (n = 10) had a mean OS of 2.65 months, whereas those who did (n = 25) had a mean OS of 23.8. This difference was shown to be statistically significant (p < 0.0001). No differences in OS were observed between single-agent (n = 2) and multi-agent (n = 20) chemotherapy (p = 0.5541). Likewise, no differences in OS between localized (n = 5) and craniospinal (n = 7) radiation (p = 0.6705) were observed.

Conclusion: Patient’s age at diagnosis and treatment with adjuvant chemoradiation are associated with greater overall survival, and no particular regimen was associated with greater survival. Further studies are required to optimize treatment strategies for ATRTs.

Publication History

Article published online:
07 February 2025