Takahashi Akira, Yamamoto Akifumi
Dermatology Division, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045, Japan.
Gan To Kagaku Ryoho. 2004 Mar;31(3):356-9.
Extramammary Paget's disease is a malignant tumor with the characteristic bright large atypical cells called Paget's cells, which proliferate within an intraepidermal lesion outside the breast. It occurs most frequently in the external genitalia, followed by perianal and axillary regions. The main clinical symptoms are erythema, erosion, pigmentation and depigmentation. Extramammary Paget's disease is commonly an intraepidermal neoplasm. However, if the tumor cells infiltrate into the dermis and form a clinically manifest nodule, it becomes an invasive carcinoma that can be classified as adnexal adenocarcinoma of the skin. Surgery with wide local excision is the first choice of treatment, and the prognosis is comparatively favorable in the early stages. However, when tumor cells infiltrate into the dermis and advance to Paget's carcinoma, it is referred to as adenocarcinoma of the skin and has a poor prognosis. We report an effective treatment of extramammary Paget's disease in advanced stages' including our case. No standard treatment with a significant effect has yet been established. There are few reports of cases responsive to chemotherapy, and treatment in advanced stages is extremely difficult at present.
乳房外佩吉特病是一种恶性肿瘤，其特征是有称为佩吉特细胞的明亮大异型细胞，这些细胞在乳房外的表皮内病变中增殖。它最常发生在外生殖器，其次是肛周和腋窝区域。主要临床症状为红斑、糜烂、色素沉着和色素脱失。乳房外佩吉特病通常是一种表皮内肿瘤。然而，如果肿瘤细胞浸润到真皮并形成临床上明显的结节，它就变成了浸润性癌，可归类为皮肤附属器腺癌。广泛局部切除手术是首选治疗方法，早期预后相对较好。然而，当肿瘤细胞浸润到真皮并发展为佩吉特癌时，就称为皮肤腺癌，预后较差。我们报告了包括我们的病例在内的晚期乳房外佩吉特病的有效治疗方法。尚未确立有显著疗效的标准治疗方法。对化疗有反应的病例报告很少，目前晚期治疗极其困难。
