胆管癌（CCA）是一类影响胆道系统的上皮癌的多样性肿瘤群。CCA在西方国家的发病率较低，但在中国和泰国等地的地方性流行区域显著较高。多种危险因素会促使CCA发生。最近的研究揭示了胆道系统癌症的分子变异，为我们了解胆管癌的发生机制和潜在的靶向治疗提供了见解。手术切除是CCA的主要治愈性治疗方法。辅助化疗已广泛研究，并有一些治疗方案被证明是有益的。新辅助化疗在特定病例中显示出潜在的益处，但其作用尚存在争议。在晚期，化疗是标准的治疗方法，并且分子分析已确认了FGFR、IDH1、HER2等潜在靶点，以及其他肿瘤不特异性治疗。在晚期CCA中，免疫疗法效果有限。本章概述了对不同阶段CCA进行各种化疗方案、靶向治疗和免疫疗法进行评估的当前证据和正在进行的研究。© 2023. 作者授权Springer Nature Switzerland AG独家使用许可。

Cholangiocarcinoma (CCA) is a diverse group of epithelial cancers that affect the biliary tree. The incidence of CCA is low in Western countries but significantly higher in endemic regions such as China and Thailand. Various risk factors contribute to the development of CCA. Recent studies have revealed molecular alterations in biliary tract cancers, providing insights into cholangiocarcinogenesis and potential targeted therapies. Surgical resection is the primary curative treatment for CCA. Adjuvant chemotherapy has been extensively studied, and some regimens have proven to be beneficial. Neoadjuvant chemotherapy has shown potential benefits in select cases, but its role remains controversial. In advanced stages, chemotherapy is the standard of care, and molecular profiling has identified potential targets such as FGFR, IDH1, HER2, and other tumor-agnostic therapies. Immunotherapy has demonstrated limited benefit in advanced CCA. This chapter provides an overview of the current evidence and ongoing research evaluating various chemotherapy regimens, targeted therapies, and immunotherapies across different stages of CCA.© 2023. The Author(s), under exclusive license to Springer Nature Switzerland AG.