Hepatocellular carcinoma in Budd-Chiari syndrome: characteristics and risk factors
Hepatocellular carcinoma in Budd-Chiari syndrome: characteristics and risk factors
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DOI：
10.1136/gut.2007.139477
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发表时间：
2008-06-01
期刊：
影响因子：
24.5
通讯作者：
Plessier, A.
中科院分区：
文献类型：
--
作者：
Moucari, R.;Rautou, P-E;Plessier, A.
背景和目标：分析Budd基亚里综合征（BCS）患者发生肝细胞癌（HCC）的特点及相关因素。根据血清甲胎蛋白（AFP）水平和影像学特征（CT/MRI）评估肝结节。结节活检时，获得以下标准之一是满足：数量= 3厘米，异质性，门静脉期洗脱，增加监测的大小，或增加AFP level.Results：患者主要是白人（69%）和女性（66%）。诊断BCS时的平均年龄为35.8岁（SE 1.2岁），中位随访时间为5年（1-20年）。下腔静脉阻塞13例。43例患者中发现肝结节，其中11例为HCC。随访期间HCC的累积发生率为4%。9例患者邻近HCC的肝实质显示肝硬化。HCC与男性（72.7%对29.0%，p = 0.007）、因子V Leiden（54.5%对17.5%，p = 0.01）和IVC梗阻（81.8%对4.6%，p < 0.001）相关。血清甲胎蛋白水平的增加是高度准确的区分肝癌良性结节：PPV = 100%和NPV = 91%的截止水平为15 ng/ml。结论：肝癌的发病率在这个大队列的BCS患者是类似的其他慢性肝病的报告。下腔静脉阻塞是肝癌发展的主要预测因素。血清甲胎蛋白似乎有更高的效用肝癌筛查BCS患者比其他肝脏疾病。
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 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.