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

Moucari, A.; Rautou, P.-E.; Cazals-Hatem, D.; Geara, A.; Bureau, C.; Consigny, Y.; Francoz, C.; Denninger, M.-H.; Vilgrain, V.; Belghiti, J.; Durand, F.; Valla, D.; Plessier, A.
June 2008
Gut;Jun2008, Vol. 57 Issue 6, p828
Academic Journal
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 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 ⩾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.


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