Although HSVN is a recently described neoplasm that has yet to be investigated, obtained data suggest that it shares similar molecular pathogenesis to other vascular malformations, such as port wine stains, tufted angioma, anastomosing, and congenital hemangioma, since mutations in these lesions are localized in the GNAQ, GNA11, and GNA14 genes [33,36,85]. This evidence concerns the gene GNAQ and vascular malformation.