Advances in the diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis
Advances in the diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis
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DOI：
10.1002/lt.20938
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发表时间：
2006-11-01
影响因子：
4.6
通讯作者：
Gores, Gregory J.
中科院分区：
文献类型：
--
作者：
Luna, Laura E. Moreno;Gores, Gregory J.
原发性硬化性胆管炎（PSC）是一种原发性慢性胆汁淤积性肝病，其特征是胆道系统的炎性破坏。它缓慢进展，导致胆道纤维化和闭塞，导致终末期肝病。PSC最可怕的并发症是胆管癌（CCA）的发展。1 PSC患者中CCA的患病率为4.8- 36.4%。2-7 PSC是英国最常见的CCA诱发因素（终身风险5-15%）。8据报道，PSC患者发生CCA的发生率为每年0.6-1.5%。9，10最高的发病率是在诊断PSC后的前2年。事实上，在37-50%患有PSC的发展CCA的患者中，恶性肿瘤的诊断是在PSC诊断后的第一年内做出的。10，11要么只有特定的PSC患者亚组有发生CCA的风险，并且在其疾病过程的早期发生，要么许多患者在就诊时有症状，难以诊断CCA。PSC患者的CCA比非PSC患者的CCA早约30年。CCA的胆管造影表现和临床症状无特异性，难以与PSC常见的良性显性胆管狭窄鉴别。13因此，PSC背景下的CCA仍然难以诊断。它通常在尸检时被发现为疾病的晚期阶段，或在肝移植时意外发现。十，十二，十四
Primary sclerosing cholangitis (PSC) is an idiopathic chronic cholestatic liver disease characterized by inflammatory destruction of the biliary tree. It slowly progresses, resulting in biliary fibrosis and obliteration leading to end-stage liver disease. The most feared complication of PSC is the development of cholangiocarcinoma (CCA). 1 The prevalence of CCA in patients with PSC ranges 4.8-36.4%. 2-7 PSC is the most common known predisposing factor for CCA in the United Kingdom (lifetime risk 5-15%). 8 It has been reported that the incidence of developing CCA in patients with PSC is 0.6-1.5% per year. 9, 10 The highest incidence is the first 2 years after the diagnosis of PSC. Indeed, in 37-50% of patients with PSC who develop CCA, the diagnosis of malignancy was made within the first year after the diagnosis of PSC. 10, 11 Either only a certain subset of patients with PSC are at risk for the development of CCA and do so early in the course of their disease, or many patients have symptomatic, difficult-to-diagnose CCA at presentation. CCA in patients with PSC has an onset about 3 decades earlier than CCA in patients without PSC. 12 The cholangiographic findings and clinical symptoms of CCA are nonspecific and are difficult to differentiate from benign dominant bile duct strictures, which are common in PSC. 13 Therefore, CCA in the setting of PSC remains difficult to diagnose. It is often found at autopsy as an advanced stage of disease, or unexpectedly at the time of liver transplantation. 10, 12, 14