Background and Aim To analyze 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 retrospectively evaluated. Liver nodules were evaluated using serum α-fetoprotein (AFP) level and imaging features (CT/MRI). Biopsy of nodules was obtained when one of the following criteria was met: number ≤3, diameter ≥3cm, 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±1.2 years, and median follow-up 5 years (1-20 years). 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 9 patients. HCC was associated with: male sex (72.7% vs. 29.0%, p=0.007); factor V Leiden (54.5% vs. 17.5% p=0.01); and IVC obstruction (81.8% vs. 4.6%, p<0.001). Increased serum AFP level had a high accuracy to distinguish HCC from benign nodules: PPV=100% and NPV=91% for a cut-off level of 15ng/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.
- Factor V Leiden
- Inferior Vena Cava Obstruction