Fibrolamellar hepatocellular carcinoma in the USA, 2000–2010: A detailed report on frequency, treatment and outcome based on the Surveillance, Epidemiology, and End Results database

OBJECTIVE: Epidemiological and clinical information on fibrolamellar hepatocellular carcinoma (fHCC) is scarce. We performed a Surveillance, Epidemiology and End Results (SEER) database analysis with the aim of collecting information to better understand the biology and clinical aspects of this rare disease. DESIGN: Incidence trends, race- and age-specific rates, tumor size, first course surgery and five-year relative survival of 191 US cases (SEER) diagnosed with fHCC during 2000-2010 were compared to cases with hepatocellular carcinoma (HCC), HCC-not otherwise specified (HCC-NOS) and other HCC-types. RESULTS: While HCC-NOS incidence rates increased by 5.2% annually from 2000-2008 (p < 0.05) before leveling, the 1.3% change in fHCC incidence was not statistically significant. The rates of fHCC were similar across ethnic groups while HCC-NOS incidence rates were higher among non-whites. Although 16% of fHCC patients had primary tumors ≤5 cm compared to 37% of HCC-NOS cases five-year survival was better among fHCC (34%) than HCC-NOS cases (16%). Fibrolamellar HCC cases of 0-39 years of age were more likely to receive radiofrequency ablation, transplant or resection than HCC-NOS cases of that age. Survival was similar among fibrolamellar and HCC-NOS cases receiving surgery. CONCLUSION: In this largest case series, fibrolamellar and HCC-NOS age- and race-specific incidence rates and time trends differed. Despite larger tumor size than HCC-NOS cases fibrolamellar cases received surgery more often and had better survival rates. Differences in co-morbidity may influence treatment. Studies of fHCC biology, including by age, are recommended.