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乳房外Paget病(EMPD)的组织发生迄今不明,它可能来源于附件腺体组织或原发于表皮。疾病主要累及大汗腺丰富的部位,如外阴、会阴、阴囊、阴茎、腋窝等部位。Chanda复习英文文献中的196例EMPD患者,有24％的病人发现相关的潜在附件腺癌,且预后不良;12％的病人在其皮肤疾病紧密相关部位伴有内脏癌。对EMPD的治疗一般多用扩大范围的局部外科切除,以防止复发,但一份研究表明,在30例原发损害用手术切除的病人中5年的复发率为40％。另外由于EMPD常发生于老年病人,常不能耐受大范围的外科手术,且发生于生殖器官的损害由于外科治疗可能致畸,患者不易接受。
The occurrence of extramural Paget disease (EMPD) is so far unknown. It may have originated from the accessory gland tissue or from the epidermis. The disease mainly affects the areas where the large sweat glands are abundant, such as the vulva, perineum, scrotum, penis, axilla, etc. Chanda reviewed 196 EMPD patients in the English literature, and 24% of patients were found to have a potential adnexal cancer associated with a poor prognosis. Twelve percent of patients had visceral cancer at sites closely related to their skin diseases. The treatment of EMPD generally uses an extended range of local surgical resection to prevent recurrence, but one study showed that the recurrence rate was 40% in 5 patients who had undergone surgical resection of primary lesions. In addition, because EMPD often occurs in elderly patients, it is often unable to tolerate a wide range of surgical operations, and the damage to reproductive organs may be teratogenic due to surgical treatment, and patients are not easy to accept.