Fibrolamellar hepatocellular carcinoma (FLC) is a usually lethal primary tumor in children, adolescents and young adults. The primary tumor is initiated and driven by a single alteration in the DNA: A deletion of ~400kb that results in a fusion gene between the heat shock co-chaperone DNAJB1 and the catalytic subunit of protein kinase A, PRKACA. If the tumor is limited to the liver, then surgery is the accepted therapy. However, if the tumor has metastasized, there is no accepted therapy. This center is focused on advancing our knowledge of how the fusion oncoprotein drives the cancer, the pathogenesis of the tumor and therapeutics that target the fusion oncoprotein. The Administrative Core is dedicated to facilitating communication and sharing of resources between the four projects, between the different investigators. Additionally, it will facilitate the flow of information and resources with the rest of the FusOnC2 consortium and the NIH.
