NCI Definition: A distinctive type of liver cell carcinoma that arises in non-cirrhotic livers and is seen predominantly in young patients. The tumor cells are polygonal and deeply eosinophilic, and are embedded in a fibrous stroma. The prognosis is similar to classical hepatocellular carcinoma that arises in non-cirrhotic livers, and better than hepatocellular carcinoma that arises in cirrhotic livers. [1]

Fibrolamellar carcinomas most frequently harbor alterations in PRKACA, DNAJB1, TERT, PREX2, and EPHA5 [2].

Most Commonly Altered Genes in Fibrolamellar Carcinoma

DNAJB1-PRKACA Fusion, PRKACA-DNAJB1 Fusion, EPHA5 Mutation, ARID1A Mutation, and ZFHX4 L1127S are the most common alterations in fibrolamellar carcinoma [2].

Top Alterations in Fibrolamellar Carcinoma

Significant Genes in Fibrolamellar Carcinoma



Disease Details

Oncocytic Hepatocellular Tumor, FLC, Hepatocellular Fibrolamellar Carcinoma, Fibrolamellar Carcinoma of the Liver Cells, Fibrolamellar Carcinoma of Liver Cells, Fibrolamellar Hepatocellular Carcinoma, Polygonal Cell Type Hepatocellular Carcinoma with Fibrous Stroma, Liver Cell Fibrolamellar Carcinoma
Hepatocellular Carcinoma
OncoTree Name
Fibrolamellar Carcinoma
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.