Balducci L, Crawford E D, Smith G F, Lambuth B, McGehee R, Hardy C
Division of Hematology Oncology, Jackson VA Medical Center, Mississippi.
Cancer Invest. 1988;6(3):293-303. doi: 10.3109/07357908809080652.
The incidence, clinical features, histogenesis, and treatment of extramammary Paget's disease (EMPD) are reviewed. This unusual skin lesion is associated with an underlying adnexal neoplasm in about 50% of cases. Also, the incidence of distant organ malignancies of EMPD is higher than expected by chance. Even in the absence of a recognizable underlying cancer, EMPD may occasionally produce distant metastases, indicating the malignant potential of this condition. Histochemical, immunohistological, and lectin binding studies demonstrate that the cell of origin of EMPD is the exocrine cell of sweat glands. Although EMPD may arise from eccrine cells, derivation from apocrine cells appears more common. The treatment of the primary lesion, by wide margin excision, is fraught by a high recurrence rate. Chemosurgery may reduce local relapse of EMPD. The value of adjuvant radiation therapy is unestablished. Chemotherapy has induced remission in 2 cases of advanced EMPD and needs testing in clinical trials.
本文综述了乳房外佩吉特病（EMPD）的发病率、临床特征、组织发生及治疗方法。这种少见的皮肤病变在约50%的病例中与潜在的附属器肿瘤相关。此外，EMPD远处器官恶性肿瘤的发生率高于偶然预期。即使在没有可识别的潜在癌症的情况下，EMPD偶尔也可能发生远处转移，表明这种疾病具有恶性潜能。组织化学、免疫组织化学和凝集素结合研究表明，EMPD的起源细胞是汗腺的外分泌细胞。虽然EMPD可能起源于小汗腺细胞，但起源于大汗腺细胞似乎更为常见。通过广泛边缘切除治疗原发性病变，复发率很高。化学外科手术可能会降低EMPD的局部复发率。辅助放疗的价值尚未确定。化疗已使2例晚期EMPD患者缓解，需要在临床试验中进行测试。
