Hepatology and Gastroenterology Section, Division of Diabetes Endocrinology and Metabolism, Department of Medicine, Imperial College London, St Mary's Hospital Campus, South Wharf Road, London, W2 1NY, UK.
Dis Model Mech. 2013 Mar;6(2):281-92. doi: 10.1242/dmm.010561.
Cholangiocarcinoma is a fatal cancer of the biliary epithelium and has an incidence that is increasing worldwide. Survival beyond a year of diagnosis is less than 5%, and therapeutic options are few. Known risk factors include biliary diseases such as primary sclerosing cholangitis and parasitic infestation of the biliary tree, but most cases are not associated with any of these underlying diseases. Numerous in vitro and in vivo models, as well as novel analytical techniques for human samples, are helping to delineate the many pathways implicated in this disease, albeit at a frustratingly slow pace. As yet, however, none of these studies has been translated into improved patient outcome and, overall, the pathophysiology of cholangiocarcinoma is still poorly understood. There remains an urgent need for new approaches and models to improve management of this insidious and devastating disease. In this review, we take a bedside-to-bench approach to discussing cholangiocarcinoma and outline research opportunities for the future in this field.
胆管癌是一种致命的胆道上皮癌，其发病率在全球范围内呈上升趋势。诊断后存活超过一年的患者不到 5%，治疗选择很少。已知的危险因素包括原发性硬化性胆管炎等胆道疾病和胆道寄生虫感染，但大多数病例与这些潜在疾病无关。许多体外和体内模型，以及用于人类样本的新型分析技术，正在帮助阐明这种疾病涉及的许多途径，尽管进展缓慢令人沮丧。然而，到目前为止，这些研究都没有转化为改善患者的预后，而且总体而言，胆管癌的病理生理学仍未得到很好的理解。仍然迫切需要新的方法和模型来改善对这种隐匿性和破坏性疾病的管理。在这篇综述中，我们采用从床边到实验室的方法来讨论胆管癌，并概述了该领域未来的研究机会。
