Recently, patients carrying heterozygous CCM3 gene mutations have been shown to be at high risk of developing meningiomas in which the wild-type allele of CCM3 is mutated (Labauge et al., 2009; Riant et al., 2013), which hints to a possible role of CCM3 as a tumor suppressor, although no mechanism for such an effect has been proposed. This evidence concerns the gene PDCD10 and meningioma.