Evaluating the efficacy of radiotherapy in patients with embryonal tumor with multilayered rosettes: A systematic review and meta-analysis.
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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 tumors with abundant neuropil and true rosettes, ependymoblastoma, and medulloepithelioma. The 5-year survival rate has been reported to be between 0% and 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 models were used to estimate survival rates between 2 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 who 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 pretreatment metastasis in the CSI group. In patients without pretreatment 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.