These carried genetic alterations related to PKA signaling, i.e. inactivating mutations in PRKAR2A (encoding the inhibitory regulatory subunit of PKA) and/or a chromosomal gain of PRKACA11, but importantly all combined with concomitant inactivating mutations of BAP1, an important tumor suppressor12–14, which is often found mutated in cholangiocarcinoma (CCA)15. This evidence concerns the gene PRKAR2A and cholangiocarcinoma.