In 2014, a new gene: LZTR1 (leucine zipper like transcription regulator 1) has been reported to be mutated in 80% of SMARCB1 mutation-negative schwannomatosis patients with somatic molecular alterations of chromosome 22q including distinct different somatic NF2 mutations as well as the loss of 22q in multiple tumors of a given patient. Here, NF2 is linked to schwannomatosis.