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Hepatocellular carcinoma in budd-chiari syndrome: characteristics and risk factors
  1. Rami Moucari (rmoucari{at}yahoo.com)
  1. Service d'Hépatologie and INSERM U773, Hôpital Beaujon, France
    1. Pierre-Emmanuel Rautou (perautou{at}yahoo.fr)
    1. Service d'Hépatologie and INSERM U773, Hôpital Beaujon, France
      1. Dominique Cazals-Hatem (dominique.cazals-hatem{at}bjn.aphp.fr)
      1. Service d'Anatomie Pathologique, Hôpital Beaujon, France
        1. Aline Geara (alinegeara{at}hotmail.com)
        1. Service d'Imagerie Médicale, Hôpital Beaujon, France
          1. Christophe Bureau (bureau.c{at}chu-toulouse.fr)
          1. Service d'Hépato Gastroentérologie, CHU de Purpan, Toulouse, France
            1. Yann Consigny (yann.consigny{at}mageos.com)
            1. Service d'Hépatologie and INSERM U773, Hôpital Beaujon, France
              1. Claire Francoz (claire.francoz{at}bjn.aphp.fr)
              1. Service d'Hépatologie and INSERM U773, Hôpital Beaujon, France
                1. Marie-Hélène Denninger (marie-helene.denninger{at}bjn.aphp.fr)
                1. Service d'Hématologie, Hôpital Beaujon, France
                  1. Valérie Vilgrain (valerie.vilgrain{at}bjn.aphp.fr)
                  1. Service d'Imagerie Médicale, Hôpital Beaujon, France
                    1. Jacques Belghiti (jacques.belghiti{at}bjn.aphp.fr)
                    1. Service de Chirurgie Digestive, Hôpital Beaujon, France
                      1. François Durand (francois.durand{at}bjn.aphp.fr)
                      1. Service d'Hépatologie and INSERM U773, Hôpital Beaujon, France
                        1. Dominique Valla (dominique.valla{at}bjn.ap-hop-paris.fr)
                        1. Service d'Hépatologie and INSERM U773, Hôpital Beaujon, France
                          1. Aurélie Plessier (aurelie.plessier{at}bjn.aphp.fr)
                          1. Service d'Hépatologie and INSERM U773, Hôpital Beaujon, France

                            Abstract

                            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.

                            • Alpha-Fetoprotein
                            • Cirrhosis
                            • Factor V Leiden
                            • Inferior Vena Cava Obstruction
                            • TIPS

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