Hepatocellular carcinoma in budd-chiari syndrome: characteristics and risk factors

Rami Moucari ¹, Pierre-Emmanuel Rautou ¹, Dominique Cazals-Hatem ², Aline Geara ³, Christophe Bureau ⁴, Yann Consigny ¹, Claire Francoz ¹, Marie-Hélène Denninger ⁵, Valérie Vilgrain ³, Jacques Belghiti ⁶, François Durand ¹, Dominique Valla ^1* and Aurélie Plessier ¹

¹ Service d'Hépatologie and INSERM U773, Hôpital Beaujon, France
² Service d'Anatomie Pathologique, Hôpital Beaujon, France
³ Service d'Imagerie Médicale, Hôpital Beaujon, France
⁴ Service d'Hépato Gastroentérologie, CHU de Purpan, Toulouse, France
⁵ Service d'Hématologie, Hôpital Beaujon, France
⁶ Service de Chirurgie Digestive, Hôpital Beaujon, France

^* To whom correspondence should be addressed. E-mail: dominique.valla{at}bjn.ap-hop-paris.fr.

Accepted 8 January 2008

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 -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.

Keywords: Alpha-Fetoprotein, Cirrhosis, Factor V Leiden, Inferior Vena Cava Obstruction, TIPS

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

Relevant Article

Digest

Robin Spiller and Magnus Simren
Gut 2008 57: 1-2. [Extract] [Full Text] [PDF]

This article has been cited by other articles:

• Valla, D-C (2008). Budd-Chiari syndrome and veno-occlusive disease/sinusoidal obstruction syndrome. Gut 57: 1469-1478 [Full Text]