Retrospective Study
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Oncol. May 24, 2022; 13(5): 352-365
Published online May 24, 2022. doi: 10.5306/wjco.v13.i5.352
Survival characteristics of fibrolamellar hepatocellular carcinoma: A Surveillance, Epidemiology, and End Results database study
Tomoki Sempokuya, Arnold Forlemu, Muaataz Azawi, Krixie Silangcruz, Nathalie Khoury, Jihyun Ma, Linda L Wong
Tomoki Sempokuya, Arnold Forlemu, Nathalie Khoury, Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE 68198, United States
Muaataz Azawi, Division of Gastroenterology and Hepatology, Sanford Center for Digestive Health, Sioux Falls, SD 57105, United States
Krixie Silangcruz, Department of Medicine, John A. Burns School of Medicine, University of Hawaii at Manoa, Honolulu, HI 96813, United States
Jihyun Ma, Department of Biostatistics, College of Public Health, University of Nebraska Medical Center, Omaha, NE 68198, United States
Linda L Wong, Department of Surgery, John A. Burns School of Medicine, University of Hawaii at Manoa, Honolulu, HI 96813, United States
Author contributions: Sempokuya T contributed to study design, data collection, statistical analysis; Sempokuya T, Forlemu A, Silangcruz K, Azawi M, Khoury N contributed to the literature review, manuscript drafting, and editing; Ma J contributed to study design and statistical analysis; Wong LL contributed to study supervision, manuscript drafting, and editing; all of the authors have approved the final version of the manuscript.
Institutional review board statement: Due to the utilization of a publicly available, de-identified database, review by our institutional review board was not required.
Informed consent statement: Informed consent was not required to conduct this study.
Conflict-of-interest statement: Wong LL is a speaker bureau for Eisai. All other authors do not have any conflicts of interest.
Data sharing statement: All of the data used in this analysis is available from the SEER database.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Tomoki Sempokuya, MD, Doctor, Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of Nebraska Medical Center, 982000 Nebraska Medical Center, Omaha, NE 68198, United States. tsempoku@hawaii.edu
Received: December 25, 2021
Peer-review started: December 25, 2021
First decision: March 16, 2022
Revised: March 29, 2022
Accepted: April 21, 2022
Article in press: April 21, 2022
Published online: May 24, 2022
Processing time: 149 Days and 13 Hours
Abstract
BACKGROUND

Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare and distinct type of hepatocellular carcinoma that frequently presents in an advanced stage in younger patients with no underlying liver disease. Currently, there is a limited understanding of factors that impact outcomes in FL-HCC.

AIM

To characterize the survival of FL-HCC by age, race, and surgical intervention.

METHODS

This is a retrospective study of The Surveillance, Epidemiology, and End Results database. We identified patients with FL-HCC between 2000-2018 by using an ICD-O-3 site code C22.0 and a histology code 8171/3: Hepatocellular carcinoma, fibrolamellar. In addition, demographics, tumor characteristics, types of surgical procedure, stages, and survival data were obtained. We conducted three separate survival analyses by age groups; ≤ 19, 20-59, and ≥ 60-year-old, and race; White, Black, Hispanic, Asian and Pacific islanders (API), and surgical types; Wedge resection or segmental resection, lobectomy, extended lobectomy (lobectomy + locoregional therapy or resection of the other lobe), and transplant. The Chi-Square test analyzed categorical variables, and continuous variables were examined using the Mann-Whitney U test. The Kaplan-Meier survival curve was used to compare survival. Multivariate analysis was done with Cox regression analysis.

RESULTS

We identified 225 FL-HCC patients with a mean age of 36.9. Overall median survival was 34 (95%CI: 27-41) mo. Patients ≤ 19-years-old had more advanced disease with positive lymph nodes status. However, they received more surgical interventions such as a wedge, segmental resection, lobectomy, extended lobectomy, and transplant. Survival for ≤ 19 was 85 (95%CI: 37-137) mo, age 20-59 was 29 (95%CI: 18-41) mo, and age ≥ 60 years was 12 (95%CI: 7-31) mo (P < 0.001). There were no differences in stage, lymph node status, metastasis status, and surgical treatment among races. The median survival were; Whites had 39 (95%CI: 29-63), Blacks 26 (95%CI: 5-92), Hispanics 31 (95%CI: 11-54), and APIs 28 (95%CI: 5-39) mo (P = 0.28). Of 225 patients, 111 FL-HCC patients had surgical procedures. Median survivals for a wedge or segmental resection was 112 (95%CI: 78-NA), lobectomy was 92 (95%CI: 57-NA), extended lobectomy was 54 (95%CI: 23-NA), and a transplant was 63 (95%CI: 20-NA) mo (P < 0.001). The median survival was better in patients who had surgical treatments regardless of lymph nodes or metastasis status (P < 0.001).

CONCLUSION

FL-HCC occurs in a primarily younger population, but survival can be prolonged despite the aggressive disease. There were no racial differences in the survival of FL-HCC; however, Asians with FL-HCC tended to be older than in other races. Surgical treatment provided better survival even in those patients with nodal disease or metastases. Although future studies are needed to explore other therapies for FL-HCC, surgical options should be considered in all cases of FL-HCC unless contraindicated.

Keywords: Fibrolamellar hepatocellular carcinoma; Transplant; Race; Age; Survival

Core Tip: Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare and distinct type of hepatocellular carcinoma. Currently, there is limited data on survival associated with FL-HCC. This retrospective study based on the Surveillance, Epidemiology, and End Results database suggests a better survival of younger patients with FL-HCC, although they had aggressive diseases. This trend may be because they received more surgical interventions. There were no racial differences in survival for FL-HCC, which is seen in HCC. The patient who had wedge or segmental resection or lobectomy had better survival.