Intrahepatic cholangiocarcinoma (ICC) is a highly lethal hepatobiliary cancer, and very few patients can undergo surgery. The prognosis of advanced ICC is poor, especially in patients who progress after first-line chemotherapy, with a median overall survival of less than 10 months. A 64-year-old male was diagnosed with advanced intrahepatic cholangiocarcinoma with ERBB2 (HER2) 3 + amplification determined by tissue-based testing and confirmed by next-generation sequencing. The patient was treated with pyrotinib added to pembrolizumab and lenvatinib after progressing with pyrotinib and tegafur and responded very well with regression of the tumor on imaging as well as normalization of tumor marker levels without serious adverse events. PET-CT after 6 months of treatment showed a partial response. The progression-free survival with second-line treatment was 17 months. For the third line of therapy, lenvatinib and pembrolizumab were used in combination with bevacizumab. Currently, he has had stable disease for approximately 6 months during third-line treatment. Adding pyrotinib to pembrolizumab and lenvatinib may represent a promising strategy for advanced ICC patients who have high levels of HER2.

中文翻译：

---

派姆单抗和乐伐替尼联合吡咯替尼治疗 HER2 阳性晚期肝内胆管癌的临床反应：病例报告

肝内胆管癌（Intrahepatic cholangiocarcinoma，ICC）是一种高致死率的肝胆癌，极少数患者可以接受手术治疗。晚期ICC预后较差，尤其是一线化疗后进展的患者，中位总生存期不足10个月。一名 64 岁男性被诊断为晚期肝内胆管癌，通过基于组织的测试确定 ERBB2 (HER2) 3 + 扩增，并通过下一代测序确认。在使用吡咯替尼和替加氟取得进展后，患者接受了帕博利珠单抗和乐伐替尼联合吡咯替尼治疗，并且对影像学上的肿瘤消退以及肿瘤标志物水平正常化反应非常好，没有严重的不良事件。治疗 6 个月后的 PET-CT 显示部分反应。二线治疗的无进展生存期为 17 个月。对于第三线治疗，lenvatinib 和 pembrolizumab 与贝伐珠单抗联合使用。目前，他在三线治疗期间病情稳定了大约 6 个月。在 pembrolizumab 和 lenvatinib 的基础上加入吡咯替尼对于 HER2 水平高的晚期 ICC 患者来说可能是一种很有前途的策略。