背景：联合性肝细胞胆管癌（cHCC-CCA）是一种高度侵袭性的恶性肿瘤，预后不良。然而，尚无共识的治疗指南，决策通常根据肝内胆管癌 (ICC) 或肝细胞癌 (HCC) 推断。鉴于 cHCC-CCA 明确存在肝细胞和胆管细胞分化，抗 PD1 抗体、多激酶抑制剂和针对这两种成分的化疗的组合方案可能是最佳选择。病例介绍：我们介绍了一名术后转移性化疗耐药性 cHCC-CCA 患者的病例，该患者对由抗 PD1 抗体信迪利单抗、多激酶抑制剂乐伐替尼和白蛋白结合型紫杉醇组成的联合疗法表现出持久的反应和合理的耐受性，尽管具有低肿瘤突变负荷 (TMB-L)、微卫星稳定性 (MSS) 和阴性程序性细胞死亡 1 配体 1 (PD-L1)。结论：免疫检查点抑制剂信迪利单抗、多激酶抑制剂乐伐替尼和白蛋白结合型紫杉醇化疗联合治疗 HCC 和 ICC 成分，可能为 cHCC-CCA 患者提供一种有前景的治疗选择。需要进一步的研究来在更大的患者群体中验证这些发现。版权所有 © 2023 Zhou、Lei、Tan、Huang、Zhang、Liang 和 Gou。

Background: Combined hepatocellular-cholangiocarcinoma (cHCC-CCA) is a highly aggressive malignancy with a poor prognosis. However, there are no consensus treatment guidelines, and decisions are usually extrapolated from intrahepatic cholangiocarcinoma (ICC) or hepatocellular carcinoma (HCC). Given that cHCC-CCA owns the unequivocal presence of both hepatocytic and cholangiocytic differentiation, a combination regimen of anti-PD1 antibody, multikinase inhibitor, and chemotherapy targeting against both components might be an optimal choice. Case presentation: We present the case of a patient with postoperative metastatic chemotherapy-resistant cHCC-CCA who exhibited a durable response and reasonable tolerability to a combination therapy consisting of the anti-PD1 antibody sintilimab, multikinase inhibitor lenvatinib, and nab-paclitaxel, despite having a low tumor mutational burden (TMB-L), microsatellite stability (MSS), and negative programmed cell death 1 ligand 1 (PD-L1). Conclusion: The combination regimen of immune checkpoint inhibitor sintilimab, multikinase inhibitor lenvatinib, and chemotherapy with nab-paclitaxel, which targets both the HCC and ICC components, may represent a promising treatment option for patients with cHCC-CCA. Further research is warranted to validate these findings in larger patient cohorts.Copyright © 2023 Zhou, Lei, Tan, Huang, Zhang, Liang and Gou.