Fibrolamellar hepatocellular carcinoma: current clinical perspectives Kelly J Lafaro,1 Timothy M Pawlik2 1Center for Pancreatic Cancer Research, Memorial Sloan Kettering Cancer Center, New York, NY, 2Department of Surgery, The Johns Hopkins University School of Medicine, Baltimore, MD, USA Abstract: Fibrolamellar carcinoma (FLC) is a variant of hepatocellular carcinoma (HCC), which comprises ~1%–9% of all HCCs. Although FLC is a variant of HCC, it is distinct from HCC in that it most often affects younger patients (10–35 years of age) with no underlying liver disease. FLC often presents with vague abdominal pain, nausea, abdominal fullness, malaise, and weight loss. Surgery is the current mainstay of treatment for FLC and remains the only potentially curative option. While FLCs are considered less responsive to chemotherapy than their classic HCC counterparts, there have been suggestions that multimodality treatments may be effective, especially in advanced cases. Further research is necessary to determine effective systemic therapies as an adjunct to surgery for FLC. Keywords: hepatocellular carcinoma, fibrolamellar, hepatocyte paraffin I, locoregional therapy