Activating mutations of PIK3CA are also observed in up to 75% of LMs, as well as combined malformations, such as Klippel-Trenaunay syndrome (KTS); congenital lipomatous overgrowth, vascular malformations, epidermal nevis, spinal/skeletal anomalies/scoliosis (CLOVES); and PIK3CA-related overgrowth spectrum (PROS) syndromes (17, 18). The gene discussed is PIK3CA; the disease is Klippel-Trénaunay syndrome.