Although mostly sporadic, PanNET can develop as a component of hereditary multi-tumor syndromes, including multiple endocrine neoplasia type 1 (MEN1), von Hippel-Lindau (VHL) disease, and, more rarely, neurofibromatosis type 1 (NF-1), and tuberous sclerosis (TSC) [6]. This evidence concerns the gene NF1 and tuberous sclerosis.