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A Systematic Review: Treatment and Prognosis of Patients with Fibrolamellar Hepatocellular Carcinoma.

Plain English summary: This paper is not available publicly.  Below is the abstract from the authors.  We are trying to obtain a copy of the paper so we can give you a plain English summary.  Based on the abstract, the authors have have examined the fate of 575 patients with Fibrolamallear Hepatocellular Carcinoma.  The survey is based both on patients in the authors' own institution, and other cases reported in the literature.  The authors conclude that partial remove of the liver (partial hepatectomy) has excellent survival rates.

Authors' Abstract

A Systematic Review: Treatment and Prognosis of Patients with Fibrolamellar Hepatocellular Carcinoma

Mavros MN, Mayo SC, Hyder O, Pawlik TM.

J Am Coll Surg. 2012 Sep 12. pii: S1072-7515(12)01034-4. doi: 10.1016/j.jamcollsurg.2012.08.001. [Epub ahead of print]

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.