Cholangiocarcinoma (CCA) is a highly lethal adenocarcinoma of the hepatobiliary system, which can be classified as intrahepatic, perihilar and distal. Each anatomic subtype has distinct genetic aberrations, clinical presentations and therapeutic approaches. In endemic regions, liver fluke infection is associated with CCA, owing to the oncogenic effect of the associated chronic biliary tract inflammation. In other regions, CCA can be associated with chronic biliary tract inflammation owing to choledocholithiasis, cholelithiasis, or primary sclerosing cholangitis, but most CCAs have no identifiable cause. Administration of the anthelmintic drug praziquantel decreases the risk of CCA from liver flukes, but reinfection is common and future vaccination strategies may be more effective. Some patients with CCA are eligible for potentially curative surgical options, such as resection or liver transplantation. Genetic studies have provided new insights into the pathogenesis of CCA, and two aberrations that drive the pathogenesis of non-fluke-associated intrahepatic CCA, fibroblast growth factor receptor 2 fusions and isocitrate dehydrogenase gain-of-function mutations, can be therapeutically targeted. CCA is a highly desmoplastic cancer and targeting the tumour immune microenvironment might be a promising therapeutic approach. CCA remains a highly lethal disease and further scientific and clinical insights are needed to improve patient outcomes.

胆管癌（CCA）是肝胆系统的一种高致死性腺癌，可分为肝内癌、肝门部癌和远端癌。每个解剖学亚型都有不同的遗传畸变、临床表现和治疗方法。在流行地区，由于相关慢性胆道炎症的致癌作用，肝吸虫感染与 CCA 相关。在其他地区，CCA 可能与胆总管结石、胆石症或原发性硬化性胆管炎引起的慢性胆道炎症有关，但大多数 CCA 没有明确的病因。服用驱虫药吡喹酮可降低肝吸虫感染 CCA 的风险，但再感染很常见，未来的疫苗接种策略可能更有效。一些 CCA 患者有资格进行潜在的治愈性手术选择，例如切除术或肝移植。遗传学研究为 CCA 的发病机制提供了新的见解，并且驱动非吸虫相关肝内 CCA 发病机制的两个畸变、成纤维细胞生长因子受体 2 融合和异柠檬酸脱氢酶功能获得性突变可以作为治疗目标。CCA 是一种高度促结缔组织增生的癌症，靶向肿瘤免疫微环境可能是一种很有前途的治疗方法。CCA 仍然是一种高度致命的疾病，需要进一步的科学和临床见解来改善患者的预后。驱动非吸虫相关肝内 CCA 发病机制的两种异常，成纤维细胞生长因子受体 2 融合和异柠檬酸脱氢酶功能获得性突变，可以作为治疗目标。CCA 是一种高度促结缔组织增生的癌症，靶向肿瘤免疫微环境可能是一种很有前途的治疗方法。CCA 仍然是一种高度致命的疾病，需要进一步的科学和临床见解来改善患者的预后。驱动非吸虫相关肝内 CCA 发病机制的两种异常，成纤维细胞生长因子受体 2 融合和异柠檬酸脱氢酶功能获得性突变，可以作为治疗目标。CCA 是一种高度促结缔组织增生的癌症，靶向肿瘤免疫微环境可能是一种很有前途的治疗方法。CCA 仍然是一种高度致命的疾病，需要进一步的科学和临床见解来改善患者的预后。