SMARCB1 and schwannomatosis: Germline SMARCB1 pathogenic variants account for up to 48% of familial cases and 10% of sporadic cases, while the germline LZTR1 pathogenic variants account for up to 38% of familial cases and 30% of sporadic cases (Boyd et al., 2008; Hadfield et al., 2008; Hutter et al., 2014; Rousseau et al., 2011; Sestini et al., 2008; Smith et al., 2015, 2014, 2012), and up to 80% of schwannomatosis cases with 22q loss in tumors and lacking a pathogenic variant in SMARCB1 (Piotrowski et al., 2014).