A phase II study of tazemetostat in 20 children with SMARCB1-deficient tumours, including malignant rhabdoid tumours, failed to meet primary endpoint with an objective response rate in only 1 child, but 6-month progression-free survival was 35%, suggesting it may have a modest effect on disease stabilisation [88]. This evidence concerns the gene SMARCB1 and rhabdoid tumor.