In contrast, the bulk of the evidence suggests that the ATRX and DAXX, and MEN-1genes are significantly mutated in most patients with highly proliferative G3 WD-pNETs, as are genes encoding key molecules of the mTOR signaling pathway (18, 19), and these genetic alterations are similar to those observed in the less malignant G1 and G2 WD-pNETs. This evidence concerns the gene ATRX and Wilson disease.