Recently KPT-9274, an inhibitor of nicotinamide phosphoribosyltransferase (NAMPT) and p21-activated kinase 4 (PAK4), was found active in pancreatic neuroendocrine tumors [12] and is currently being evaluated in a human phase 1 study in patients with advanced solid malignancies. This evidence concerns the gene PAK4 and pancreatic neuroendocrine tumor.