Yamazaki N, Yamamoto A, Wada T, Ishikawa M, Moriya Y, Nakanishi Y
Dermatology Division, National Cancer Center Hospital, Tokyo, Japan.
J Dermatol. 1999 May;26(5):311-6. doi: 10.1111/j.1346-8138.1999.tb03477.x.
Extramammary Paget's disease is considered to be a malignant tumor originating from the sweat glands. Some cases of extramammary Paget's disease infiltrate the dermis and metastasize to the regional lymph nodes. No standard treatment has been established for advanced cases. Few previous studies of the treatment for metastatic Paget's disease have revealed an effective regimen. The prognosis of metastasized cases is very poor. We encountered a patient in whom extramammary Paget's disease had metastasized to the lymph nodes beyond the regional lymph nodes and systemic chemotherapy was partially effective. Combination chemotherapy consisted of mitomycin C 3.5 mg/m2 and epirubicin 50 mg/m2 on day 1, vincristine 0.6 mg/m2 on days 1 and 7, cisplatin 30 mg/m2 from days 1 to 3, and 5-fluorouracil 350 mg/m2 from days 3 to 7. After two courses of chemotherapy, the metastatic lymph nodes decreased in size by more than 90% compared to that before chemotherapy. We defined the treatment effect of this regimen as a partial response (PR). Microscopic examination of the resected lymph nodes revealed replacement of metastatic lesions by fibrous tissue, suggesting a therapeutic effect. Anorexia, alopecia, and leukopenia (neutropenia) have been reported as toxicities, but all were tolerated. Our results may provide useful indications for the management of this tumor. This particular combination chemotherapy is recommended for extramammary Paget's disease patients with systemic nodal metastases.
乳腺外佩吉特病被认为是一种起源于汗腺的恶性肿瘤。部分乳腺外佩吉特病病例会浸润真皮并转移至区域淋巴结。对于晚期病例，尚未确立标准治疗方案。既往关于转移性佩吉特病治疗的研究很少揭示出有效的治疗方案。转移病例的预后非常差。我们遇到一名患者，其乳腺外佩吉特病已转移至区域淋巴结以外的淋巴结，全身化疗有部分疗效。联合化疗方案为第1天给予丝裂霉素C 3.5 mg/m²和表柔比星50 mg/m²，第1天和第7天给予长春新碱0.6 mg/m²，第1至3天给予顺铂30 mg/m²，第3至7天给予5-氟尿嘧啶350 mg/m²。两个化疗疗程后，转移淋巴结的大小较化疗前缩小了90%以上。我们将该方案的治疗效果定义为部分缓解（PR）。对切除淋巴结的显微镜检查显示转移性病变被纤维组织取代，提示有治疗效果。据报道，厌食、脱发和白细胞减少（中性粒细胞减少）为毒性反应，但所有这些反应患者均可耐受。我们的结果可能为该肿瘤的治疗提供有用的参考。对于有全身淋巴结转移的乳腺外佩吉特病患者，推荐使用这种特定的联合化疗方案。
