Focal versus craniospinal radiation for disseminated atypical teratoid/rhabdoid tumor following favorable response to systemic therapy

Purpose Radiotherapy (RT) is associated with improved survival in atypical teratoid/rhabdoid tumor (ATRT); however, optimal RT delivery is unknown. A meta‐analysis was conducted for disseminated (M+) ATRT receiving focal or craniospinal radiation (CSI). Methods After screening, 25 studies (1995–2020) contained necessary patient, disease, and radiation treatment information (N = 96). All , full text, and data capture were independently double‐reviewed. The corresponding author was contacted for cases of insufficient information. Response to pre‐radiation chemotherapy (N = 57) was categorized as complete response (CR), partial response (PR), stable disease (SD), and progressive disease (PD). Univariate and multivariate statistics were performed to investigate survival correlation. Patients with M4 disease were excluded. Results The 2‐ and 4‐year overall survival (OS) was 63.8% and 45.7%, respectively, with a median follow‐up of 2 years (range 0.3–13.5). The median age was 2 years (range 0.2–19.5), and 96% received chemotherapy. On univariate analysis, gross total resection (GTR, p = .0007), pre‐radiation chemotherapy response (p