PRKACA and fibrolamellar hepatocellular carcinoma: PRKACA defects have also been found in cardiac myxomas, and chromosomal PRKACA rearrangements were identified in fibrolamellar hepatocellular carcinoma and in intraductal oncocytic papillary neoplasms of the pancreas and bile duct, along with PRKACB defects (126–128). Inactivating somatic defects in PRKAR1A as well as activating somatic defects in GNAS have also been described in CPAs with a prevalence of 5%, and 4.5% to 11%, respectively (60, 65).