A larger proportion of germline mutations are found in sporadic pancreatic neuroendocrine tumors (PanNETs) than in other NETs in genes such as the DNA repair genes MUTYH, CHEK2, and BRCA2, affecting processes such as chromatin remodeling, activation of mTOR signaling (including previously undescribed EWSR1 gene fusions), telomere maintenance, hypoxia and HIF signaling (Scarpa et al., 2017). This evidence concerns the gene CHEK2 and pancreatic neuroendocrine tumor.