PDF(Pubmed)
Abstract：
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要：
■乳腺外Paget病（EMPD）是一种罕见的上皮恶性肿瘤，约30%-40%的EMPD患者过表达人表皮生长因子受体2(Her-2)。目前,对于晚期EMPD尚无既定的标准治疗方法,而对于Her-2阳性病例则建议使用抗Her-2治疗.
■这里，我们报告了一名51岁的男性，诊断为晚期Her-2阳性EMPD，表现为许多淋巴结转移。该患者接受了diitamabvedotin（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，患者对治疗的耐受性良好，淋巴结病变继续缩小。然而,患者在第8次治疗后出现免疫疗法相关肺炎.在停止所有抗肿瘤治疗的同时施用激素治疗。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,显示他的肿瘤完全有反应.为了巩固效果，他又接受了五个周期的diitamabvedotin单药治疗作为维持治疗，没有经历任何不良事件。迄今为止,停药10个月后,患者健康状况良好,无复发.
■Disitamabvedotin联合免疫治疗在晚期Her-2阳性EMPD中显示出长期临床益处。对于Her-2过度表达的罕见实体瘤，二他单抗vedotin联合免疫治疗可能提供可行的治疗选择.
■这里，我们报告了一名51岁的男性，诊断为晚期Her-2阳性EMPD，表现为许多淋巴结转移。该患者接受了diitamabvedotin（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，患者对治疗的耐受性良好，淋巴结病变继续缩小。然而,患者在第8次治疗后出现免疫疗法相关肺炎.在停止所有抗肿瘤治疗的同时施用激素治疗。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,显示他的肿瘤完全有反应.为了巩固效果，他又接受了五个周期的diitamabvedotin单药治疗作为维持治疗，没有经历任何不良事件。迄今为止,停药10个月后,患者健康状况良好,无复发.
■Disitamabvedotin联合免疫治疗在晚期Her-2阳性EMPD中显示出长期临床益处。对于Her-2过度表达的罕见实体瘤，二他单抗vedotin联合免疫治疗可能提供可行的治疗选择.
