Neurofibromatosis type III

(schwannomatosis)


The association of a mutation of the SMARCCB1 and the LZTR1 genes can produce schwannomatosis. The proteins encoded by these genes are tumor suppressors. It seems that local somatic mutations are also necessary for schwannomatosis to develop: the most frequent are mutations of the NF2 gene or a deletion of the chromosome 22 (on which the SMARCB1, LZTR1 and NF2 genes are located).


Anesthetic implications:

according to the localization of the tumors


References : 


Updated: October 2022