Cholangiocarcinoma: advances in pathogenesis, diagnosis, and treatment.
Cholangiocarcinoma: advances in pathogenesis, diagnosis, and treatment.
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胆管癌：发病机理，诊断和治疗的进展。
DOI：
10.1002/hep.22310
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发表时间：
2008-07
期刊：
影响因子：
13.5
通讯作者：
Gores, Gregory J.
中科院分区：
文献类型：
--
作者：
Blechacz, Boris;Gores, Gregory J.
胆管癌（Cholangiocarcinoma，CCA）是一种起源于胆管的上皮性肿瘤，具有胆管细胞分化的特征。1 CCA是第二常见的原发性肝脏恶性肿瘤，流行病学研究表明其发病率在西方国家呈上升趋势。2晚期CCA预后极差，中位生存期为24个月。3唯一的治愈性治疗是手术摘除或肝移植，但不幸的是，大多数患者患有晚期疾病，这不适合手术治疗。在解剖学上，CCA分为肝外和肝内两种形式。肝外型更常见，占CCA的80%至90%。根据肿瘤在肝外胆道系统中的位置，可进一步分为近端或肝门周围和远端亚组。肝门周围疾病也经常被称为Klatskin肿瘤。肝外胆管癌有三种不同的生长方式：（1）导管周围浸润，（2）乳头状或导管内生长，（3）肿块形成。4肝内CCA通常表现为肝内肿块。除了它们不同的形态学和临床表现外，肝内和肝外CCA在发病机制、分子特征和治疗方面也不同。在过去的几年里，有显着的新见解的分子发病机制的CCA。还开发了新的诊断和治疗模式，提高了检测率和结果。此外，我们现在已经进入了人类癌症靶向治疗的时代。因此，这是及时和局部审查这些进展，重点是有前途的靶向治疗这种疾病。另一个目标是激发对这种疾病的进一步兴趣，希望改善这种仍然高度致命的恶性肿瘤的结局。
Cholangiocarcinoma (CCA) is an epithelial can-cer originating from the bile ducts with features of cholangiocyte differentiation. 1 CCA is the second most common primary hepatic malignancy, and epidemiologic studies suggest its incidence is increasing in Western countries. 2 Advanced CCA has a devastating prognosis, with a median survival of 24 months. 3 The only curative therapy is surgical extirpation or liver transplantation, but unfortunately the majority of patients present with advanced stage disease, which is not amenable to surgical therapies. Anatomically, CCA is classified into extrahepatic and intrahepatic forms of the disease. The extrahepatic form is more common, accounting for 80% to 90% of CCAs. It is further divided into proximal or perihilar and distal subsets depending on the location of the cancer within the extrahepatic biliary system. Perihilar disease is also frequently referred to as a Klatskin tumor. Three different growth patterns of extrahepatic CCA can be observed:(1) periductal infiltrating,(2) papillary or intraductal, and (3) mass forming. 4 Intrahepatic CCA typically presents as an intrahepatic mass. In addition to their distinct morphology and clinical presentations, intrahepatic and extrahepatic CCAs differ in etiopathogenesis, molecular signatures, and management. In the last several years there have been significant new insights into the molecular pathogenesis of CCA. New diagnostic and therapeutic modalities have also been developed, resulting in improved detection rates and outcomes. In addition, we have now entered the era of targeted therapies for human cancers. Therefore, it is timely and topical to review these advances with a focus on promising targeted therapies for this disease. An additional goal is to stimulate further interest in this disease with the hope of improving outcomes for this still highly lethal malignancy.
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影响因子：
24.5
作者：
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