Abstract
BACKGROUND: Cholangiocarcinoma (CCA) poses a significant clinical challenge due to its low radical resection rate and a propensity for high postoperative recurrence, resulting in a poor dismal. Although the combination of targeted therapy and immunotherapy has demonstrated notable efficacy in several solid tumors recently, however, its application in CCA remains underexplored and poorly documented. CASE SUMMARY: This case report describes a patient diagnosed with stage IV CCA, accompanied by liver and abdominal wall metastases, who underwent palliative surgery. Subsequently, the patient received two cycles of treatment combining lenvatinib with sintilimab, which resulted in a reduction in abdominal wall metastasis, while intrahepatic metastasis displayed progression. This unexpected observation illustrates different responses of intrahepatic and extrahepatic metastases to the same therapy. CONCLUSION: Lenvatinib combined with sintilimab shows promise as a potential treatment strategy for advanced CCA. Genetic testing for related driver and/or passenger mutations, as well as an analysis of tumor immune microenvironment analysis, is crucial for optimizing drug combinations and eventually addressing the issue of non-response in specific metastatic sites.