摘要肝内胆管癌（ICC）是发病率居第2位的原发性肝癌，近年来该病的发病率上升。ICC通常起病隐匿，导致确诊较晚。目前肝切除是ICC唯一被证实有效的根治性治疗措施，但切除率低，远期疗效不理想。局部治疗联合系统性化疗是目前晚期或不可切除ICC的主要治疗方法，但效果较差。随着对ICC基因表达谱研究的深入及二代测序技术的进展，多个异常信号转导通路（RAS/MAPK、MET、EGFR）及基因突变（FGFR2、IDH1/2）等潜在治疗靶点相继被发现。虽然目前尚无针对ICC的靶向药物获得批准使用，但已有百余项应用范围包括ICC的靶向药物单用或联合化疗的临床试验，部分显示良好的应用前景。随着ICC分子分型研究的进展，个体化的靶向治疗可能是ICC新的治疗突破点。

abstractsIntrahepatic cholangiocarcinoma(ICC) is the second most common primary liver cancer. The incidence of ICC has been significantly increased globally in recent years. The concealed onset of ICC usually results in late disease diagnosis. Liver resection is currently the only well-established treatment for ICC that may cure the disease, however, long-term survival rate is still unsatisfied due to the low resection rate and high recurrence rate. Local therapy combined with systemic chemotherapy is the main treatment for advanced or unresectable ICC, but the outcomes are still poor. With the in-depth understanding of the molecular mechanism of ICC and development of next-generation sequencing technology, multiple abnormal signaling pathways (RAS/MAPK, MET, EGFR) and gene mutations (FGFR2, IDH1/2) have been identified as potential therapeutic targets. Although there is still no approved targeted drugs for ICC, more than 100 clinical trials testing targeted therapy alone or in combination with chemotherapy are ongoing, among which some have shown promising application prospects. Molecular typing and personalized targeted therapy are important ways to improve the overall outcomes of ICC. This review summarized the recent advances in the targeted therapies for patients with ICC.