A subdivision of tumors with Ki67 > 20% into G3 WD-NETs or G3 PD-NECs was proposed in the 2017 WHO classification for neuroendocrine neoplasms of pancreatic origin [14], leading to the identification of a new category comprising WD tumor morphology and Ki67 index > 20%, referred to as G3 pNETs. Here, MKI67 is linked to Wilson disease.