Hepatocellular carcinoma in Budd-Chiari syndrome: characteristics and risk factors

Background and aim: To analyse the characteristics of and the factors associated with the development of hepatocellular carcinoma (HCC) in patients with Budd Chiari syndrome (BCS). Patients and methods: 97 consecutive patients with BCS and a follow-up >= 1 year were evaluated retrospectively. Liver nodules were evaluated using serum a-fetoprotein (AFP) level and imaging features (CT/MRI). Biopsy of nodules was obtained when one of the following criteria was met: number <= 3, diameter >= 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. Conclusion: 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.