The majority of GISTs are driven by sporadic somatic KIT or PDGFRA mutations rather than inflammation-associated carcinogenesis; rare hereditary and syndromic forms of GIST (e.g., familial KIT/PDGFRA mutations, Carney–Stratakis syndrome, and neurofibromatosis type 1–associated GIST) do exist [8,9,10]. This evidence concerns the gene KIT and neurofibromatosis type 1.