Associated Genetic Biomarkers
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. 
Fibrolamellar carcinomas most frequently harbor alterations in PRKACA, DNAJB1, TERT, PREX2, and EPHA5 .
DNAJB1-PRKACA Fusion, PRKACA-DNAJB1 Fusion, EPHA5 Mutation, ARID1A Mutation, and ZFHX4 L1127S are the most common alterations in fibrolamellar carcinoma .
There are 2 clinical trials for fibrolamellar carcinoma, of which 2 are open and 0 are completed or closed. Of the trials that contain fibrolamellar carcinoma as an inclusion criterion, 1 is phase 1 (1 open) and 1 is phase 1/phase 2 (1 open).
DNAJB1-PRKACA is the most frequent gene inclusion criterion for fibrolamellar carcinoma clinical trials .
Nivolumab, dnajb1-prkaca peptide vaccine, and fluorouracil are the most common interventions in fibrolamellar carcinoma clinical trials.
Significant Genes in Fibrolamellar Carcinoma
DNAJB1 is altered in 89.29% of fibrolamellar carcinoma patients .
DNAJB1 is an inclusion eligibility criterion in 1 clinical trial for fibrolamellar carcinoma, of which 1 is open and 0 are closed. Of the trial that contains DNAJB1 status and fibrolamellar carcinoma as inclusion criteria, 1 is phase 1 (1 open) .
PRKACA is altered in 89.29% of fibrolamellar carcinoma patients .
PRKACA is an inclusion eligibility criterion in 1 clinical trial for fibrolamellar carcinoma, of which 1 is open and 0 are closed. Of the trial that contains PRKACA status and fibrolamellar carcinoma as inclusion criteria, 1 is phase 1 (1 open) .
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.