Although most cases (90% of cases) arise as isolated/solitary lesions (Figure 10 and Figure 11), their occurrence within the context of NF2-related-schwannomatosis or NF2/MERLIN schwannoma predisposing syndrome (formerly, NF2), SMARCB1/LZTR1-related-schwanomatoses or SMARCB1/LZTR1 schwannoma predisposing syndromes (formerly, schwannomatosis) and 22q-related-schwannomatosis or 22q schwannoma predisposing syndrome (Table 7) is well-known [1,8]. Here, SMARCB1 is linked to schwannomatosis.