Abstract

This liver tumor in a young adult was diagnosed by pathology as fibrolamellar carcinoma. What is different about fibrolamellar carcinoma compared with conventional hepatocellular carcinoma?a.Fibrolamellar carcinoma usually affects patients in their sixth and seventh decadesb.Fibrolamellar carcinoma almost always occurs in a background of liver cirrhosisc.Fibrolamellar carcinoma is strongly associated with DNAJB1-PRKACA oncogenic fusiond.Fibrolamellar carcinoma almost exclusively occurs in females Answer: c. Fibrolamellar carcinoma is strongly associated with DNAJB1-PRKACA oncogenic fusion Fibrolamellar carcinoma is an uncommon malignancy, comprising 1% to 5% of liver cancers. This tumor occurs in younger patients, usually less than 40 years of age, and affects males and females. Conventional hepatocellular carcinoma most often affects older adults. Most conventional hepatocellular carcinomas occur in livers affected by cirrhosis, while fibrolamellar carcinoma occurs in noncirrhotic livers. Fibrolamellar carcinoma is strongly associated with DNAJB1-PRKACA oncogenic fusions, which were also recently reported in intraductal oncocytic neoplasms of the pancreas and bile duct.1Honeyman J.N. Simon E.P. Robine N. et al.Detection of a recurrent DNAJB1-PRKACA chimeric transcript in fibrolamellar hepatocellular carcinoma.Science. 2014; 343: 1010-1014Crossref PubMed Scopus (292) Google Scholar,2Singhi A.D. Wood L.D. Parks E. et al.Recurrent rearrangements in PRKACA and PRKACB in intraductal oncocytic papillary neoplasms of the pancreas and bile duct.Gastroenterology. 2020; 158: 573-582Abstract Full Text Full Text PDF PubMed Scopus (66) Google Scholar

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