Successful treatment of a case of hormone receptor?positive metastatic extramammary Paget disease with tamoxifen
Successful treatment of a case of hormone receptor?positive metastatic extramammary Paget disease with tamoxifen
复制标题
他莫昔芬成功治疗激素受体阳性转移性乳房外佩吉特病一例
DOI：
10.1007/s10637-021-01168-5
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发表时间：
2021
影响因子：
3.4
通讯作者：
Nakagawa Kazuhiko
中科院分区：
文献类型：
--
作者：
Isomoto Kohsuke;Haratani Koji;Watanabe Satomi;Takeda Masayuki;Iwasa Tsutomu;Nakagawa Kazuhiko
乳房外佩吉特病（EMPD）是一种罕见的皮肤腺癌，通常起源于表皮，并显示出腺体分化，根据疾病的程度通过广泛的局部切除治疗。然而，对于广泛转移性疾病，标准治疗仍有待建立。与乳腺癌类似，EMPD被发现过表达人表皮生长因子受体2（HER2）或激素受体（HR）。尽管最近显示HER2导向疗法对HER2阳性EMPD有效，但内分泌疗法对HR阳性EMPD的潜在作用仍未知。我们在此报告一例转移性EMPD伴HR阳性，经选择性雌激素受体调节剂他莫昔芬成功治疗。这种全身转移的一线治疗导致持久的肿瘤消退> 20个月，而没有任何治疗相关的毒性。这是第一份报告，揭示了他莫昔芬作为一种安全有效的治疗HR阳性转移性EMPD的承诺。
Extramammary Paget disease (EMPD) is a rare cutaneous adenocarcinoma that usually is of epidermal origin and shows glandular differentiation and that is treated by wide local excision depending on the disease extent. For widely metastatic disease, however, a standard treatment remains to be established. Similar to breast cancer, EMPD has been found to overexpress human epidermal growth factor receptor 2 (HER2) or hormone receptors (HRs). Whereas HER2-directed therapy was recently shown to be effective for HER2-positive EMPD, the potential role of endocrine therapy for HR-positive EMPD has remained unknown. We here report a case of metastatic EMPD with HR positivity that was successfully treated with the selective estrogen receptor modulator tamoxifen. This first-line treatment of systemic metastasis resulted in durable tumor regression for > 20 months without any treatment-related toxicities. This is the first report to reveal the promise of tamoxifen as a safe and effective treatment for HR-positive metastatic EMPD.