Wang Dongxing, Huang Chuang, Wang Dongming, Chang Dehui
Department of Urology, The 940t Hospital of Joint Logistics Support Force of Chinese People's Liberation Army, Lanzhou, Gansu, China.
Department of Dermatology, Shuguang Hospital Affiliated to Shanghai University of Traditional Chinese Medicine, Shanghai, China.
Front Oncol. 2024 Oct 24;14:1402490. doi: 10.3389/fonc.2024.1402490. eCollection 2024.
Extramammary Paget disease (EMPD) is a rare epithelial adenocarcinoma in apocrine-gland rich skin, involving the vulva, the scrotum, and the penis. with distant metastases and a poor prognosis. Local EMPD patients generally have a good prognosis, with expected 5-year survival of 60%-92%, but distant metastasis represents poor prognosis and 5-year survival of 10%. Treatment approaches for advanced EMPD are chemotherapy and biological agents, which carry limited efficacy. We report the case of a 57-year-old man diagnosed with metastatic EMPD, who showed a long-term disease control with a combination therapy (an immune checkpoint inhibitor - tislelizumab plus chemotherapy - paclitaxel albumin and cisplatin). This patient underwent a wide penile scrotal lesion excision and six cycles of tislelizumab plus chemotherapy. The patient achieved partial response for the metastatic lesions according to the Response Evaluation Criteria in Solid Tumors (version 1.1). This case report supports further investigation of the combination treatment of chemotherapy and immune checkpoint inhibitors in the management of metastatic EMPD, which currently has an abysmal prognosis and no standardized treatment.
乳房外佩吉特病（EMPD）是一种发生于富含顶泌汗腺皮肤的罕见上皮腺癌，累及外阴、阴囊和阴茎，可发生远处转移且预后较差。局部EMPD患者一般预后良好，预计5年生存率为60% - 92%，但远处转移则预后不良，5年生存率为10%。晚期EMPD的治疗方法为化疗和生物制剂，疗效有限。我们报告了一例57岁男性转移性EMPD患者，其通过联合治疗（免疫检查点抑制剂 - 替雷利珠单抗加化疗 - 白蛋白结合型紫杉醇和顺铂）实现了长期疾病控制。该患者接受了广泛的阴茎阴囊病变切除术及六个周期的替雷利珠单抗联合化疗。根据实体瘤疗效评价标准（第1.1版），该患者的转移性病变达到部分缓解。本病例报告支持进一步研究化疗与免疫检查点抑制剂联合治疗转移性EMPD，目前转移性EMPD预后极差且尚无标准化治疗方案。
