To summarize, pancreatic NETs, generally seen as a frequent type of neuroendocrine neoplasia among other sites, are associated with a genetic background in one out of ten cases, mostly MEN1 but also MEN4 and other syndromes such as neurofibromatosis type 1, Cowden syndrome, von Hippel–Lindau disease, etc. [108,143,144,145]. This evidence concerns the gene MEN1 and pancreatic neuroendocrine tumor.