原发性尿道癌（PUC）发病率低，但侵袭性高。多数患者发现时已属晚期，预后较差。目前化疗仍是转移性PUC的主要治疗方法，但效果有限。在这里，我们报告一例 HER2 表达低的转移性 PUC，在化疗、程序性死亡 1 (PD-1) 抑制剂和多靶点受体酪氨酸激酶 (RTK) 抑制剂等多线治疗后出现疾病进展。在接受Disitamab Vedotin（一种新型抗体药物偶联物，ADC）和特瑞普利单抗（一种PD-1抑制剂）治疗后，患者实现了持续PR，迄今为止PFS超过12个月。我们的报告表明，尽管转移性PUC患者HER2表达较低，但Disitamab Vedotin联合PD-1抑制剂仍有可能受益，可能在一定程度上逆转PD-1抑制剂和化疗的耐药性。但需要更大样本的研究来确定这种治疗策略的疗效及其对生存的影响。版权所有 © 2023 Cheng、Xue、Zhao、Chen 和 Li。

Primary urethral carcinoma (PUC) has a low incidence, but with high aggressiveness. Most of the patients are found in late stage, with poor prognosis. At present, chemotherapy is still the main treatment for metastatic PUC, but it has limited effect. Here, we report a case of metastatic PUC with low HER2 expression that developed disease progression after multiline therapy including chemotherapy, programmed death-1 (PD-1) inhibitors and multi-targeted receptor tyrosine kinase (RTK) inhibitor. After receiving Disitamab Vedotin(a novel antibody drug conjugate, ADC) and toripalimab (a PD-1 inhibitor), the patient achieved persistent PR, and the PFS exceeded 12 months up to now. Our report indicates that, despite the patient of metastatic PUC has low expression of HER2, it is still possible to benefit from Disitamab Vedotin combined with PD-1 inhibitor, which may reverse the drug resistance of PD-1 inhibitor and chemotherapy to a certain extent. But larger sample studies are needed to determine the efficacy of this treatment strategy and its impact on survival.Copyright © 2023 Zheng, Xue, Zhao, Chen and Li.