Fibrolamellar Hepatocellular Carcinoma (FL-HCC) Epidemiology, Survival Characteristics, and Outcomes: Surveillence, Epidemiology, and End Results Database (SEER) Study

Document Type

Conference Proceeding

Publication Date

6-1-2024

Publication Title

Journal of Clinical Oncology

Abstract

Background: Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare and distinctive form of liver cancer that primarily affects adolescents and young adults without underlying liver disease. Unlike conventional HCC, it exhibits unique clinical and histological features and poses a diagnostic and therapeutic challenge. Using a national registry, we sought to evaluate the incidence, demographics, survival, and treatment characteristics of FL-HCC. Methods: We utilized the Surveillance, Epidemiology, and End Results (SEER) database to identify patients with microscopically confirmed FL-HCC between 2000-2020 using ICD-O-3 site code C22.0 and an 8171/3 histology code. The epidemiological characteristics included were age, gender, ethnicity, American Joint Committee on Cancer [AJCC] staging, annual income, and treatment modalities. The Kaplan-Meier survival curve was used to compare survival. Survival was stratified according to age, gender, race, stage, income, and treatment modalities. Multivariate analysis was done with Cox regression analysis on R programming language version 3.6.3. Results: We identified a total of 362 patients with fibrolamellar hepatocellular carcinoma with median age at diagnosis 27(8-84) years. It was found to be more common in males (62.2%) and among whites (78.2%). There was a striking increment in incidence with 1.9% in 2005 compared to 5.8% in 2020. The majority of the patients had stage IV disease (26.4%). Alpha-fetoprotein was noted to be positive only in 15.8% of patients. The majority of the patients received surgery (50.3%) and chemotherapy (48.1%) with a median time from diagnosis to treatment of 1.00 month (IQR: 0.00–1.00). A total of 11% of patients received radiation, and 25% received multiple treatments. Survival analysis revealed a median overall survival of 24.50 months (IQR: 7.00, 63.00), with a majority experiencing death attributable to the cancer diagnosis (60.2%). Median overall and cause-specific survival for stage I disease was 92 months, and stage IV disease was 21 months (p < 0.05). Patients below the age of 20 had a median survival of 85 months (95% CI: 41 to NA) compared to those aged between 20 and 59, i.e., 30 months (95% CI: 22 to 41 months). The higher-income categories, $55,000−59,999 (HR = 0.29, p = 0.05) and $60,000−64,999 (HR = 0.24, p = 0.03), demonstrated significantly lower hazards compared to < $35,000. We concluded that sex, location, and race did not influence the survival of FL-HCC. Conclusions: Our comprehensive analysis of FL-HCC revealed that diagnosis at a younger age, stage I disease, and higher annual income improved survival, whereas gender and race did not affect outcomes. Further collaborative efforts are warranted to advance our understanding of FL-HCC pathogenesis, optimize treatment strategies, and improve outcomes for affected individuals.

Volume

42

Issue

16 Suppl

First Page

e16213

Comments

American Society of Clinical Oncology Annual Meeting, ASTRO 2024, May 31 - June 4, 2024, Chicago, IL

DOI

10.1200/JCO.2024.42.16_suppl.e16213

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