Zhang Xiaoting, Jin Wanwan, Zhu Haibo, Yu Haifeng
Department of Dermatology, The second Affiliated Hospital and Yuying children's hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325000, People's Republic of China.
Department of Urology, The second Affiliated Hospital and Yuying children's hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325000, People's Republic of China.
Indian J Dermatol. 2015 Jul-Aug;60(4):423. doi: 10.4103/0019-5154.160541.
Extramammary Paget's disease (EMPD), which is considered to be an adenocarcinoma of the apocrine glands, is a rare, slow-growing neoplasm. The standard treatment for local EMPD is surgical resection, however, so far, no effective treatment is found for advanced EMPD. Trastuzumab, which is a recombinant monoclonal antibody against the human epidermal growth factor receptor 2 HER2 has been proved to be effective in the treatment of advanced EMPD in some cases where HER2 protein is overexpressed. Herein, we report two cases of EMPD in two brothers. The younger brother who presented as local EMPD on the scrotum received surgical resection and had no recurrence in 15 months following. The older brother suffered from invasive EMPD also on the scrotum with inguinal region multiple metastatic lymph nodes and was treated with combination chemotherapy and Trastuzumab to target HER2 consecutively after a wide surgical excision.
乳腺外佩吉特病（EMPD）被认为是一种大汗腺腺癌，是一种罕见的、生长缓慢的肿瘤。局部EMPD的标准治疗方法是手术切除，然而，到目前为止，尚未发现针对晚期EMPD的有效治疗方法。曲妥珠单抗是一种针对人表皮生长因子受体2（HER2）的重组单克隆抗体，已被证明在某些HER2蛋白过度表达的晚期EMPD病例中治疗有效。在此，我们报告两兄弟患EMPD的病例。弟弟表现为阴囊局部EMPD，接受了手术切除，术后15个月未复发。哥哥也患有阴囊浸润性EMPD，伴有腹股沟区多发转移性淋巴结，在广泛手术切除后连续接受了联合化疗和针对HER2的曲妥珠单抗治疗。
