Now, based on mutational studies, CD34 negativity and presence of isolated or double-hit MYC rearrangement [152, 153], both WHO-HAEM5 and ICC recommend to consider these cases as DLBCL or HGBCL, NOS with “expression of TdT” (Fig. 4). This evidence concerns the gene MYC and intrahepatic cholangiocarcinoma.