The majority of NETs are sporadic, but they can also occur in the context of inherited familial syndromes, such as multiple endocrine neoplasia type 1 (MEN1), Von-Hippel Lindau syndrome, tuberous sclerosis and neurofibromatosis type 1, thus suggesting a causal role for genetic alterations during the tumorigenic process [1]. This evidence concerns the gene MEN1 and multiple endocrine neoplasia type 1.