A systematic review: Treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma

Michael N. Mavros, Skye Mayo, Omar Hyder, Timothy M. Pawlik

Research output: Contribution to journalArticle

62 Citations (Scopus)

Abstract

Background: Fibrolamellar hepatocellular carcinoma (FLC) is a rare primary liver tumor presenting earlier in life than nonfibrolamellar hepatocellular carcinoma (NFL-HCC), with distinct epidemiologic and clinical characteristics. Although FLC is believed to have a better prognosis than NFL-HCC, data on treatment and prognosis are scarce. We performed a systematic review to investigate treatment options and clinical outcomes of patients with FLC. Study Design: The study is a systematic review of the literature and pooled analysis of individual patient data. Results: A total of 35 series were analyzed, reporting on 575 patients (52% female, elevated alpha-fetoprotein in 10%, cirrhosis in 3%, hepatitis B in 2%), most of whom were treated with partial hepatectomy (55%) or orthotopic liver transplantation (23%). Nineteen studies provided data on 206 individual patients with a median age of 21 years and tumor size of 12 cm. Median overall survival (OS) was 39 months; 1-year, 3-year, and 5-year OS rates were 85%, 53%, and 44%, respectively. For patients treated with liver resection, median OS was 18.5 years and 1-year, 3-year, and 5-year OS were 93%, 80%, and 70%, respectively. Based on data from 15 studies, FLC appeared to follow a relatively indolent course compared with NFL-HCC. Conclusions: Patients with FLC treated with partial hepatectomy have excellent long-term survival, with 5-year overall survival reaching 70%. Patients fared worse with the use of other therapeutic options including chemotherapy, intra-arterial therapy, and transplantation, although data directly comparing resection vs transplantation were limited.

Original languageEnglish (US)
Pages (from-to)820-830
Number of pages11
JournalJournal of the American College of Surgeons
Volume215
Issue number6
DOIs
StatePublished - Dec 2012
Externally publishedYes

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Survival
Hepatocellular Carcinoma
Hepatectomy
Therapeutics
Transplantation
Liver
alpha-Fetoproteins
Therapeutic Uses
Fibrolamellar hepatocellular carcinoma
Hepatitis B
Liver Transplantation
Neoplasms
Fibrosis
Survival Rate
Drug Therapy

ASJC Scopus subject areas

  • Surgery

Cite this

A systematic review : Treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma. / Mavros, Michael N.; Mayo, Skye; Hyder, Omar; Pawlik, Timothy M.

In: Journal of the American College of Surgeons, Vol. 215, No. 6, 12.2012, p. 820-830.

Research output: Contribution to journalArticle

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abstract = "Background: Fibrolamellar hepatocellular carcinoma (FLC) is a rare primary liver tumor presenting earlier in life than nonfibrolamellar hepatocellular carcinoma (NFL-HCC), with distinct epidemiologic and clinical characteristics. Although FLC is believed to have a better prognosis than NFL-HCC, data on treatment and prognosis are scarce. We performed a systematic review to investigate treatment options and clinical outcomes of patients with FLC. Study Design: The study is a systematic review of the literature and pooled analysis of individual patient data. Results: A total of 35 series were analyzed, reporting on 575 patients (52{\%} female, elevated alpha-fetoprotein in 10{\%}, cirrhosis in 3{\%}, hepatitis B in 2{\%}), most of whom were treated with partial hepatectomy (55{\%}) or orthotopic liver transplantation (23{\%}). Nineteen studies provided data on 206 individual patients with a median age of 21 years and tumor size of 12 cm. Median overall survival (OS) was 39 months; 1-year, 3-year, and 5-year OS rates were 85{\%}, 53{\%}, and 44{\%}, respectively. For patients treated with liver resection, median OS was 18.5 years and 1-year, 3-year, and 5-year OS were 93{\%}, 80{\%}, and 70{\%}, respectively. Based on data from 15 studies, FLC appeared to follow a relatively indolent course compared with NFL-HCC. Conclusions: Patients with FLC treated with partial hepatectomy have excellent long-term survival, with 5-year overall survival reaching 70{\%}. Patients fared worse with the use of other therapeutic options including chemotherapy, intra-arterial therapy, and transplantation, although data directly comparing resection vs transplantation were limited.",
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