Road map for fibrolamellar carcinoma: progress and goals of a diversified approach

Abstract

Edward R Kastenhuber,1,2 John Craig,3 Jon Ramsey,4 Kevin M Sullivan,5 Julien Sage,6,7 Sofia de Oliveira,8 Kimberly J Riehle,5 John D Scott,5 John D Gordan,9 Nabeel Bardeesy,10,11 Ghassan K Abou-Alfa1,12 1Cancer Biology and Genetics Program, Memorial Sloan Kettering Cancer Center, New York, NY, USA; 2Louis V. Gerstner Jr. Graduate School of Biomedical Sciences, Memorial Sloan Kettering Cancer Center, New York, NY, USA; 3Fibrolamellar Cancer Foundation, Greenwich, CT, USA; 4Department of Biochemistry, University of Vermont Cancer Center, Burlington, VT, USA; 5Northwest Liver Research Program, University of Washington, Seattle, WA, USA; 6Department of Pediatrics, Stanford University, Stanford, CA, USA; 7Department of Genetics, Stanford University, Stanford, CA, USA; 8Department of Medical Microbiology and Immunology, University of Wisconsin, Madison, WI, USA; 9Department of Medicine, University of California, San Francisco, San Francisco, CA, USA; 10Center for Cancer Research, Massachusetts General Hospital, Boston, MA, USA; 11Department of Medicine, Harvard Medical School, Boston, MA, USA; 12Department of Medicine, Weill Cornell School of Medicine, New York, NY, USA Abstract: Fibrolamellar carcinoma is a rare liver cancer, which primarily afflicts adolescents and young adults worldwide and is frequently lethal. Given the rarity of this disease, patient recruitment for clinical trials remains a challenge. In November 2017, the Second Fibrolamellar Cancer Foundation Scientific Summit (Stamford, CT, USA) provided an opportunity for investigators to discuss recent advances in the characterization of the disease and its surrounding liver and immune context. The Fibrolamellar Cancer Foundation has thus set out a road map to identify and test therapeutic targets in the most efficient possible manner. Keywords: fibrolamellar carcinoma, liver cancer, adolescent, young adult, fusion oncogene, cyclic AMP-dependent protein kinase catalytic subunit, heat shock protein 40

Keywords

• Fibrolamellar carcinoma (FLC)
• DNAJB1-PRKACA fusion