In this criteria, the “NF2” term was updated to “NF2-related schwannomatosis”, and the previous “schwannomatosis” became updated per the relevant pathogenic variant: SMARCB1-related schwannomatosis, LZTR1-related schwannomatosis, 22q-related schwannomatosis, schwannomatosis-NOS (not otherwise specified), or schwannomatosis NEC (not elsewhere classified) [76]. This evidence concerns the gene SMARCB1 and schwannomatosis.