title: A systematic review: treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma.
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.
  
  
  METHODS
  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.
authors.affiliation: Department of Surgery, Johns Hopkins University School of Medicine, 600 N Wolfe St, Baltimore, MD 21287, USA.
authors.forename: Michael N
authors.initials: MN
authors.lastname: Mavros
authors.forename: Skye C
authors.initials: SC
authors.lastname: Mayo
authors.forename: Omar
authors.initials: O
authors.lastname: Hyder
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Pawlik
mesh_terms: D006528:Carcinoma, Hepatocellular / Q000401:mortality / Q000628:therapy*; D003131:Combined Modality Therapy; D014943:Global Health; D006801:Humans; D008113:Liver Neoplasms / Q000401:mortality / Q000628:therapy*; D011379:Prognosis; D015996:Survival Rate / Q000639:trends