title: Treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma: a national perspective.
abstract: BACKGROUND
  Surgery remains the only potentially curative option for patients with hepatocellular carcinoma (HCC) and fibrolamellar carcinoma (FLC). We sought to investigate the differences over time in surgically managed FLC compared with conventional HCC using population-based data.
  
  
  METHODS
  Using SEER data, we identified 7,225 patients with surgically managed FLC or HCC from 1986 to 2008. We examined differences in clinicopathologic and surgical factors associated with long-term survival.
  
  
  RESULTS
  Of the 7,225 patients, the majority had HCC (n = 7,135; 99%) vs FLC (n = 90; 1%). Patients with FLC were younger (25 years vs 59 years) and more often were women (44% vs 27%) than patients with HCC (both p < 0.001). Regional disease was more common among patients with FLC (42.2%) vs patients with HCC (22.1%) (p < 0.001). More than one-third of patients with FLC (36.9%) were operatively managed with a hemihepatectomy compared with patients with HCC, who were more often managed with a liver transplant (p < 0.001). On univariable analysis, there was a marked difference in overall survival, with patients with FLC surviving a median of 75 months vs 43 months for HCC (hazard ratio [HR]: 0.59; p = 0.001). There was a marked difference in survival when patients were stratified by localized (FLC, 78 months vs HCC, 49 months; p = 0.012) vs regional disease (FLC, 46 months vs HCC, 23 months; p = 0.002.
  
  
  CONCLUSIONS
  Patients with FLC have many clinicopathologic features that are different from those of patients with HCC, including younger age and female sex. Despite a higher likelihood of advanced disease at the time of diagnosis, surgically treated FLC patients had better long-term outcomes than patients with conventional HCC.
authors: Department of Surgery, Johns Hopkins Hospital, Baltimore, MD.
authors: Skye C
authors: SC
authors: Mayo
authors: Department of Surgery, Johns Hopkins Hospital, Baltimore, MD; Department of Surgery, MedStar Washington Hospital Center, Washington, DC.
authors: Michael N
authors: MN
authors: Mavros
authors: Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY.
authors: Hari
authors: H
authors: Nathan
authors: Department of Medical Oncology, Johns Hopkins Hospital, Baltimore, MD.
authors: David
authors: D
authors: Cosgrove
authors: Department of Radiology, Johns Hopkins Hospital, Baltimore, MD.
authors: Joseph M
authors: JM
authors: Herman
authors: Department of Radiation Oncology, Johns Hopkins Hospital, Baltimore, MD.
authors: Ihab
authors: I
authors: Kamel
authors: Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD.
authors: Robert A
authors: RA
authors: Anders
authors: Department of Surgery, Johns Hopkins Hospital, Baltimore, MD. Electronic address: tpawlik1@jhmi.edu.
authors: Timothy M
authors: TM
authors: Pawlik
mesh_terms: D000328:Adult; D006528:Carcinoma, Hepatocellular / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D006498:Hepatectomy / Q000379:methods*; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009017:Morbidity / Q000639:trends; D009367:Neoplasm Staging; D011379:Prognosis; D012189:Retrospective Studies; D018426:SEER Program; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D014481:United States / Q000453:epidemiology
keywords: FLC; HCC; HR; NCI; National Cancer Institute; OS; fibrolamellar carcinoma; hazard ratio; hepatocellular carcinoma; overall survival