Four different inherited syndromes are associated with the development F-PNETs; patients with Multiple Endocrine Neoplasia type 1(MEN1); von Hippel Lindau Disease (VHL); von Recklinghausen disease (VRH) (neurofibromatosis 1) (NF-1) and patients with tuberous sclerosis (13). This evidence concerns the gene NF1 and von Hippel-Lindau disease.