Similarly, other data suggest that 17% of patients with seemingly sporadic pancreatic neuroendocrine tumors harbor germline alterations in any one of a variety of genes (including MUTYH, CHEK2, and BRCA2, as well as MEN1 and VHL), suggesting that all patients with glucagonoma should at least be considered for inherited genetic syndromes testing (including, but not limited to, MEN1) [41]. This evidence concerns the gene VHL and pancreatic neuroendocrine tumor.