Interestingly, these aberrations distribution is according to the anatomic location: in ICC are found mostly mutations in IDH1/2, BAP1, and ARID1A and FGFR rearrangements, while in ECC are more frequently found novel fusions in PRKACA/PRKACB and mutations in ARID1B (see Fig. 1) [37]. Here, BAP1 is linked to intrahepatic cholangiocarcinoma.