Recently, Evans et al. integrated large databases of over 2700 individuals with molecular testing for NF2.23 The authors suggested that the terms “glioma” and “neurofibroma” be removed and replaced by “ependymoma” and that testing of the gene LZTR1 be recommended for individuals with unilateral VS given clinical overlap between LZTR1-associated schwannomatosis and NF2. This evidence concerns the gene LZTR1 and central nervous system cancer.