Fibrolamellar Hepatocellular Carcinoma: A Population-Based Observational Study

Abstract

Background

In the USA, fibrolamellar hepatocellular carcinoma (FLC) accounts for 1–2% of all cases of hepatocellular carcinoma. FLC remains poorly understood.

Aim

We aim to investigate the incidence, demographics, tumor characteristics, treatment, and prognosis of patients with FLC.

Methods

Data on FLC between 2000 and 2016 were extracted from the SEER database and analyzed.

Results

A total of 300 patients with FLC were identified where 126 were male. Median age at diagnosis was 27 ± 22 years. The overall age-adjusted incidence of FLC between 2000 and 2016 was 0.02 per 100,000 per year. A bimodal distribution was observed where the highest incidences occurred between 15–19 years and 70–74 years. Most tumors on presentation were moderately differentiated (20.7%), while the most common stage at presentation was stage 1 (21.7%) followed by stages 3 and 4 (20.0% and 20.3%, respectively); 50.3% of these tumors were surgically resected, while 8.0% received radiation and 45.3% received chemotherapy. One- and 5-year cause-specific survival for FLC was 72.0% and 32.9%, respectively, with a median survival of 32.9 months. HCC had a median survival time of 11.7 months. Patients who were not treated with surgical intervention had about 3 times increased risk for death (HR 2.8, 95% CI 1.68–4.72, P = 0.000). Radiation and chemotherapy did not significantly affect outcomes.

Conclusion

FLC presents with a bimodal distribution in both early and elderly individuals. Compared to HCC, FLC has a higher recurrence rate but better survival outcome. Surgical intervention is superior to chemotherapy and radiation.

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Authors

Contributions

DR conceived the study and conducted data analysis; DR, JL, and AO interpreted the data and drafted the manuscript. ZHG provided histology. DA and ZHG revised the manuscript for intellectual content. All authors approved the final version of the manuscript.

Corresponding author

Correspondence to Douglas G. Adler.

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Ramai, D., Ofosu, A., Lai, J.K. et al. Fibrolamellar Hepatocellular Carcinoma: A Population-Based Observational Study. Dig Dis Sci 66, 308–314 (2021). https://doi.org/10.1007/s10620-020-06135-3

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Keywords

  • Hepatocellular carcinoma
  • Fibrolamellar hepatocellular carcinoma
  • Surveillance
  • Epidemiology and End Results Registry
  • Resection
  • Prognosis