Certain mutations in KLKB1, mutated in one of five CT-pNENs, cause prekallikrein deficiency (56), and KLKB1 was mutually exclusive with ATRX, DAXX, and MEN1 of pancreatic neuroendocrine tumors (PanNets) (57, 58). This evidence concerns the gene DAXX and Congenital prekallikrein deficiency.