Volume 148, Issue 4, Pages 707-710 (April 2015) Fibrolamellar Hepatocellular Carcinoma: A Rare but Distinct Type of Liver Cancer  Jesper B. Andersen  Gastroenterology  Volume 148, Issue 4, Pages 707-710 (April 2015) DOI: 10.1053/j.gastro.2015.02.039 Copyright © 2015 Terms and Conditions

Figure 1 Disease progression in liver cancer. Fibrolamellar carcinoma (FLC) most often occurs on the background of a normal-appearing, noncirrhotic liver. Recent genetic studies have shown a high prevalence of a fusion gene, DNAJB1-PRKACA, in 79%–100% of patients diagnosed with FLC. Whereas cholangiocarcinoma (CCA) and hepatocellular carcinoma (HCC) and their pathologic subtypes (such as scirrhous HCC or mixed CCA-HCC) as well as the individual genetic variants typically present with an overall poor prognosis, patients diagnosed with FLC usually have a better prognosis. This complex molecular heterogeneity further necessitates a personalized approach to therapy, highlighting the need for future genomic studies. The FLC classification shows enrichment of known gene signatures (“proliferation” and “inflammation” classes) that are also found in HCC and CCA. The “unannotated” subgroup is enriched in general cancer-related genes, such as MAPK, PIK3, RAS, CTNNB1 and TGFb, not specific to liver cancers. Gastroenterology 2015 148, 707-710DOI: (10.1053/j.gastro.2015.02.039) Copyright © 2015 Terms and Conditions