The ICC classification, as well as theELN2022 guidelines, has introduced a nine-gene signature (ASXL1, BCOR, EZH2, RUNX1, SF3B1, SRSF2, STAG2, U2AF1, and ZRSR2) to define the new category of AML (>20% blasts) or MDS/AML (10–19% blasts) with myelodysplasia-related mutations [2,47]. This evidence concerns the gene EZH2 and intrahepatic cholangiocarcinoma.