Pancreatic NENs may be sporadic or arise in the context of an inherited syndrome such as multiple endocrine neoplasia type 1 (MEN1) or type 4 (MEN4), von Hippel‒Lindau (VHL) disease, neurofibromatosis type I (NF1), and tuberous sclerosis [5]. Here, CDKN1B is linked to neurofibromatosis type 1.