Literature data suggest somatic mutations in the PI3K/AKT/mTOR pathway primarily in patients with lymphatic and venous VAs and in those with syndromic conditions (Klippel–Trénaunay syndrome, congenital lipomatous overgrowth, vascular malformations, epidermal nevi and scoliosis/skeletal/spinal anomalies (CLOVES) syndrome, blue rubber bleb nevus syndrome, etc.)(4). This evidence concerns the gene MTOR and blue rubber bleb nevus.