乳房外Paget病的治疗进展

Treatments of extramammary Paget＇s disease

乳房外Paget病好发于老年人，多发生于肛门及外生殖器周围，由于早期皮损与湿疹、皮炎类似，容易误诊。乳房外Paget病的首选治疗方法为手术治疗，由于皮损边界不清、有多处病灶、肿瘤组织呈跳跃性生长，很难保证切除干净致术后复发率高。其他治疗方法有：放射治疗、化学治疗、光动力疗法、免疫调节剂治疗、分子靶向治疗等。乳房外Paget病预后主要与肿瘤的浸润深度、区域淋巴结转移、淋巴血管转移相关。前哨淋巴结活检对淋巴结清扫有指导意义，选择性淋巴结清扫有助于后续治疗，提高患者生存率。

Extramammary Paget＇s disease （EMPD） mostly occurs in the elderly with predilection for the anogenital region. Since its early lesions are similar to eczema and dermatitis, it is easy to misdiagnose. The first- choice treatment of EMPD is surgical excision. However, EMPD is difficult to completely remove and recurrence is common because it often has muhiple lesions with obscure margins and extends beyond the clinically visible margin. Other effective treatments include radiotherapy, chemotherapy, photodynamic therapy, immunomodulatory therapy, molecular targeted therapy, and so on. The prognosis of EMPD is mainly associated with the depth of tumor invasion, local lymph node metastasis, lymphatic and vascular metastases. Sentinel lymph node biopsy can serve as a guide to lymph node dissection, and selective prophylactic lymphadenectomy is helpful for subsequent treatment with an increase in survival rates.