乳房外佩吉特病 (EMPD) 是一种罕见的上皮恶性肿瘤，大约 30%-40% 的 EMPD 患者过度表达人表皮生长因子受体 2 (Her-2)。目前，晚期 EMPD 尚无既定的标准治疗方法，但建议对 Her-2 阳性病例进行抗 Her-2 治疗。在此，我们报告一名被诊断患有晚期 Her-2 阳性 EMPD 的 51 岁男性，表现为伴有大量淋巴结转移。该患者接受 disitamab vedotin（一种抗体药物偶联物，靶向 Her-2）联合 serplulimab 作为一线治疗。经过七个周期的联合治疗后，患者对治疗的耐受性良好，淋巴结病灶持续缩小。然而，患者在第八次治疗后出现了免疫治疗相关肺炎。在停止所有抗肿瘤治疗的同时进行激素治疗。肺炎好转后，患者接受了正电子发射断层扫描-计算机断层扫描，显示肿瘤已完全缓解。为了巩固疗效，他又接受了五个周期的 disitamab vedotin 单药治疗作为维持治疗，没有出现任何不良事件。迄今为止，患者健康状况良好，停药10个月后未出现任何复发。Disitamab vedotin联合免疫治疗在晚期Her-2阳性EMPD中显示出长期临床获益。对于 Her-2 过表达的罕见实体瘤，disitamab vedotin 联合免疫疗法可能提供可行的治疗选择。版权所有 © 2024 Wang、Meng、Hu 和 Liu。

Extramammary Paget's disease (EMPD) is a rare epithelial malignancy, and approximately 30%-40% of EMPD patients overexpress human epidermal growth factor receptor 2 (Her-2). Currently, there are no established standard treatments for advanced EMPD while anti-Her-2 therapy is recommended for Her-2-positive cases.Here, we report a 51-year-old male diagnosed with advanced Her-2-positive EMPD, presenting with numerous lymph node metastases. This patient received disitamab vedotin (an antibody-drug conjugate, targeting Her-2) combined with serplulimab as first-line treatment. After seven cycles of combination therapy, the patient tolerated the treatment well and the lymph node lesions continued to shrink. However, the patient developed immunotherapy-related pneumonia following the eighth treatment. Hormone therapy was administered while all the anti-tumor therapies were halted. After the pneumonia improved, the patient underwent positron emission tomography-computed tomography, revealing a complete response to his tumor. To consolidate the effect, he received another five cycles of disitamab vedotin monotherapy as maintenance therapy, without experiencing any adverse events. To date, the patient has remained in good health without any recurrence 10 months after drug discontinuance.Disitamab vedotin combined with immunotherapy demonstrated a long-term clinical benefit in advanced Her-2-positive EMPD. For rare solid tumors with Her-2 overexpression, disitamab vedotin combined with immunotherapy might offer a viable therapeutic choice.Copyright © 2024 Wang, Meng, Hu and Liu.