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Meta-Analysis
. 2023 Apr;27(4):705-715.
doi: 10.1007/s11605-023-05621-z. Epub 2023 Feb 16.

Treatment and Prognosis of Fibrolamellar Hepatocellular Carcinoma: a Systematic Review of the Recent Literature and Meta-analysis

Dajana Glavas  1 , Quoc Riccardo Bao  1 , Marco Scarpa  1 , Cesare Ruffolo  1 , Zachary J Brown  2 , Timothy M Pawlik  2 , Gaya Spolverato  3
Affiliations
Meta-Analysis

Treatment and Prognosis of Fibrolamellar Hepatocellular Carcinoma: a Systematic Review of the Recent Literature and Meta-analysis

Dajana Glavas et al. J Gastrointest Surg. 2023 Apr.

Abstract

Background: Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare disease and current efforts are focused on the prognosis and on the development of efficient and specific treatments. This study aimed to review the latest evidence regarding FL-HCC treatment and prognosis.

Methods: A systematic review of the literature over the past 10 years regarding FL-HCC, and meta-analysis of 1-, 3-, and 5-year overall survival (OS) comparing FL-HCC and conventional HCC were performed.

Results: Overall, 1567 articles were screened, of them 21 were selected for the systematic review, and 6 for meta-analysis. Twenty-one studies included a total of 2168 patients with FL-HCC, with a median age ranging from 11 to 56 years. The majority of patients underwent surgical resection or liver transplantation. After a median follow-up ranging from 24 to 58 months, 1-year OS was 67-100% and 5-year OS was 28-65%. A total of 743 patients with FL-HCC and 163,472 with conventional HCC were included in the meta-analysis. There was a significantly improved 1-, 3-, and 5-years OS in the FL-HCC group compared to the conventional HCC group, although high heterogeneity was found. When excluding population-based studies, and including 96 FL-HCC and 221 conventional HCC patients, the heterogeneity was low, and the meta-analysis showed a significantly longer 1-year OS in patients with FL-HCC than conventional HCC; however, there were no differences at 3- and 5-years OS.

Conclusions: Surgical resection for FL-HCC is currently the only curative treatment available. FL-HCC is plagued by high-recurrence rates and poor long-term outcomes which may be related to the absence of specific treatment for advanced and recurrent disease.

Keywords: Fibrolamellar hepatocellular carcinoma; Hepatocellular carcinoma; Liver cancer.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
PRISMA flowchart of study selection
Fig. 2
Fig. 2
Meta-analysis of overall survival; a 1-year OS, b 3-years OS, c 5-years OS, d 5-years OS in resected patients
Fig. 3
Fig. 3
Meta-analysis of overall survival including retrospective studies only; a 1-year OS, b 3-years OS, c 5-years OS
See this image and copyright information in PMC

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Supplementary concepts