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

作者：Mavros Michael N; Mayo Skye C; Hyder Omar; Pawlik Timothy M^*

来源：Journal of the American College of Surgeons, 2012, 215(6): 820-830.

DOI：10.1016/j.jamcollsurg.2012.08.001

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. (J Am Coll Surg 2012;215: 820-830.

• 出版日期2012-12