The occurrence of either malignant rhabdoid tumors or schwannomatosis in families with germline SMARCB1 mutations48,49 and the occurrence of schwannomatosis in a patient that survived a malignant rhabdoid tumor50 support the idea that mutations in both RTPS1 and schwannomatosis are loss-of-function mutations of different severity but with an overlapping phenotypic spectrum. This evidence concerns the gene SMARCB1 and schwannomatosis.