Most NETs are sporadic with unknown risk factors whereas rare cases are related to inherited syndromes including multiple endocrine neoplasia (MEN) 1 and 2, von Hippel–Lindau (VHL) disease, tuberous sclerosis (TSC) complex and neurofibromatosis (NF) 1 [5,6,7]. This evidence concerns the gene NFASC and tuberous sclerosis.