Evaluating the efficacy of radiotherapy in patients with embryonal tumor with multilayered rosettes: A systematic review and meta-analysis

Abstract

Background Embryonal tumor with multilayered rosettes (ETMR) is a rare and deadly pediatric central nervous system tumor often seen before the age of 3. ETMR consists of embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma. The five-year survival rate has been reported to be between 0-30%. Treatment of ETMR is very unstandardized and typically consists of surgical resection, chemotherapy, and radiotherapy. A systematic review was performed to better understand treatment-related outcome trends. Methods The authors performed a PRISMA guidelines-based systematic review of the literature. Survival curve analysis using Kaplan-Meier curves and Cox proportional hazards (PH) models were used to estimate survival rates between two groups and multiple risk factors, respectively. Results The average survival time was 31.1 months in patients treated with radiotherapy compared to 11.2 months in patients who did not. Radiotherapy was a significant covariate on overall survival (p <.001) with an 82% lower risk of death compared to patients that did not receive radiotherapy. The average survival time for patients with focal radiotherapy was 35.8 months compared to 29.8 months in patients with CSI radiotherapy, but there was a great number of patients with pre-treatment metastasis in the CSI group. In patients without pre-treatment metastasis, focal radiotherapy had non-inferior outcomes for survival rates and times. Conclusions Patients treated with radiotherapy in addition to chemotherapy demonstrated a significantly higher survival time. For patients with no metastasis prior to treatment, focal radiotherapy should be strongly considered.