Effect of Treatment Modality on Overall Survival of Pediatric Atypical Teratoid/rhabdoid Tumor Patients: A Systematic Review and Meta-analysis

Introduction: Atypical teratoid/rhabdoid (AT/RT) tumors are a type of CNS cancer that mainly affect children < 3 years of age. Although rare (accounting for 1%-2% of pediatric CNS tumors), AT/RTs are deadly, with a survival rate of 15%-40%. The current standard of treatment involves a multimodal approach consisting of surgery, chemotherapy (CT) and radiotherapy (RT), however these therapies carry significant risk and there is no established protocol for treatment of these tumors. The purpose of this study is to systematically evaluate the overall survival of pediatric AT/RT patients treated with surgical resection, CT, and/or RT.

Methods: An article search following PRISMA guidelines was performed using PubMed, Embase, Scopus, Cochrane, Web of Science, and Globus Medicus Index libraries. Search terms included (atypical teratoid rhabdoid tumor) and (atypical (teratoid OR rhabdoid) tumor). Variables of interest included, but were not limited to, age, sex, tumor location, treatment modality, extent of resection, and overall survival.

Results: After screening, 228 studies were included for a total of 936 patients. The mean age of the cohort was 36.99 +/- 43.71 months and 56.8% were male. The overall survival of patients receiving surgery was 109.9 months compared to 21.10 months in those without surgery. Gross total resection had a longer overall survival than subtotal resection (p < 0.0001). Surgery with RT and CT had a longer overall survival than patients who had surgery with CT but no RT (p < 0.0001), but there was no significant difference between Surgery with RT and CT and surgery with RT but no CT (p=0.057).

Conclusion : Surgical resection is a mainstay treatment for patients with CNS AT/RTs, specifically with GTR. Results suggest that surgical resection combined with CT and RT may be the most effective treatment when compared to surgery with CT only; however, surgery with RT may also be as effective.