肝内胆管癌（ICC）是第二常见的原发性肝肿瘤，大多数患者的预后仍然很糟。约20%到30%的患者有资格接受手术切除治疗，这被认为是唯一潜在的治愈方案。在手术切除后，与辅助用卡培他滨的序贯治疗相结合，报道了中位生存期为53个月。对于70%-80%的患者，他们呈现为局部不能手术切除或远处转移的疾病，系统治疗可延缓疾病进展，但生存期仍然只有大约1年左右。十年来，如今珠葡二联疗法已被认为是最有效的一线治疗方案，但三联疗法甚至是免疫治疗的最新研究结果可能会改变这一模式。更有效的治疗战略，包括将系统治疗与局部区域治疗（如放射性微粒治疗或肝动脉灌注）相结合，也已经发展出来了。分子治疗，包括针对成纤维细胞生长因子受体和异柠檬酸脱氢酶的治疗，最近已获得美国食品和药物管理局的批准，作为二线治疗的一个明确定义的作用来帮助40%的患者，这是否应该在一线治疗中考虑正被研究中。此外，随着肿瘤学领域寻求扩大免疫治疗的适应症，最近的数据表明，将durvalumab与标准的细胞毒性治疗结合使用可以改善ICC患者的生存率。这篇综述着重于ICC治疗的当前和未来策略，包括每种治疗方式的主要文献综述和可用于推动个性化和多学科治疗方法的算法。©2022年作者。《肿瘤学期刊》由Wiley Periodicals LLC代表美国癌症协会出版。

Intrahepatic cholangiocarcinoma (ICC) is the second most common primary liver tumor and remains a fatal malignancy in the majority of patients. Approximately 20%-30% of patients are eligible for resection, which is considered the only potentially curative treatment; and, after resection, a median survival of 53 months has been reported when sequenced with adjuvant capecitabine. For the 70%-80% of patients who present with locally unresectable or distant metastatic disease, systemic therapy may delay progression, but survival remains limited to approximately 1 year. For the past decade, doublet chemotherapy with gemcitabine and cisplatin has been considered the most effective first-line regimen, but results from the recent use of triplet regimens and even immunotherapy may shift the paradigm. More effective treatment strategies, including those that combine systemic therapy with locoregional therapies like radioembolization or hepatic artery infusion, have also been developed. Molecular therapies, including those that target fibroblast growth factor receptor and isocitrate dehydrogenase, have recently received US Food and Drug Administration approval for a defined role as second-line treatment for up to 40% of patients harboring these actionable genomic alterations, and whether they should be considered in the first-line setting is under investigation. Furthermore, as the oncology field seeks to expand indications for immunotherapy, recent data demonstrated that combining durvalumab with standard cytotoxic therapy improved survival in patients with ICC. This review focuses on the current and future strategies for ICC treatment, including a summary of the primary literature for each treatment modality and an algorithm that can be used to drive a personalized and multidisciplinary approach for patients with this challenging malignancy.© 2022 The Authors. CA: A Cancer Journal for Clinicians published by Wiley Periodicals LLC on behalf of American Cancer Society.