For pancreatic NEN G3, differences between NET G3 and NEC have been established on a molecular level: similar to NET G1/G2, pancreatic NET G3 harbor alterations in the MEN1, DAXX/ATRX and mTOR genes, on the other hand, TP53 and RB1 mutations are almost exclusively detected in NEC and not in NET G3 [2,3,13,14]. This evidence concerns the gene ATRX and pancreatic neuroendocrine neoplasm.