The only risk factor of PNETs seems to be a genetic factor found in patients with family history of NETs or those with certain familial syndromes such as multiple endocrine neoplasia type I (MEN1), von Hippel-Lindau, tuberculous sclerosis, and neurofibromatosis type I. Nonetheless, the patients in this group usually develop tumor at early age [8]. Here, MEN1 is linked to neurofibromatosis type 1.