title: Hepatocellular carcinoma in Budd-Chiari syndrome: characteristics and risk factors.
abstract: OBJECTIVE
  To analyse the characteristics of and the factors associated with the development of hepatocellular carcinoma (HCC) in patients with Budd-Chiari syndrome (BCS).
  
  
  METHODS
  97 consecutive patients with BCS and a follow-up > or = 1 year were evaluated retrospectively. Liver nodules were evaluated using serum alpha-fetoprotein (AFP) level and imaging features (CT/MRI). Biopsy of nodules was obtained when one of the following criteria was met: number < or = 3, diameter > or = 3 cm, heterogeneity, washout on portal venous phase, increase in size on surveillance, or increase in AFP level.
  
  
  RESULTS
  Patients were mainly Caucasian (69%) and female (66%). Mean age at the diagnosis of BCS was 35.8 (SE 1.2 years), and median follow-up 5 years (1-20 years). The inferior vena cava (IVC) was obstructed in 13 patients. Liver nodules were found in 43 patients, 11 of whom had HCC. Cumulative incidence of HCC during follow-up was 4%. Liver parenchyma adjacent to HCC showed cirrhosis in nine patients. HCC was associated with male sex (72.7% v 29.0%, p = 0.007); factor V Leiden (54.5% v 17.5%, p = 0.01); and IVC obstruction (81.8% v 4.6%, p < 0.001). Increased levels of serum AFP were highly accurate in distinguishing HCC from benign nodules: PPV = 100% and NPV = 91% for a cut-off level of 15 ng/ml.
  
  
  CONCLUSIONS
  The incidence of HCC in this large cohort of BCS patients was similar to that reported for other chronic liver diseases. IVC obstruction was a major predictor for HCC development. Serum AFP appears to have a higher utility for HCC screening in patients with BCS than with other liver diseases.
authors.affiliation: AP-HP, Hôpital Beaujon, Service d'Hépatologie, Clichy, France.
authors.forename: R
authors.initials: R
authors.lastname: Moucari
authors.forename: P-E
authors.initials: PE
authors.lastname: Rautou
authors.forename: D
authors.initials: D
authors.lastname: Cazals-Hatem
authors.forename: A
authors.initials: A
authors.lastname: Geara
authors.forename: C
authors.initials: C
authors.lastname: Bureau
authors.forename: Y
authors.initials: Y
authors.lastname: Consigny
authors.forename: C
authors.initials: C
authors.lastname: Francoz
authors.forename: M-H
authors.initials: MH
authors.lastname: Denninger
authors.forename: V
authors.initials: V
authors.lastname: Vilgrain
authors.forename: J
authors.initials: J
authors.lastname: Belghiti
authors.forename: F
authors.initials: F
authors.lastname: Durand
authors.forename: D
authors.initials: D
authors.lastname: Valla
authors.forename: A
authors.initials: A
authors.lastname: Plessier
mesh_terms: D000328:Adult; D000465:Algorithms; D001706:Biopsy; D006502:Budd-Chiari Syndrome / Q000150:complications*; D006528:Carcinoma, Hepatocellular / Q000175:diagnosis / Q000209:etiology*; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis / Q000209:etiology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012737:Sex Factors; D000509:alpha-Fetoproteins / Q000378:metabolism
chemical_list: D000509:alpha-Fetoproteins