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目的:探讨外阴Paget病的临床特点及治疗方法。方法:回顾分析5例外阴Paget病的临床资料。结果:外阴Paget病5例,年龄45~75岁,平均67.4岁。4例绝经。5例中,3例(60%)以瘙痒为首发症状,2例(40%)因外阴结节、皮损或湿疹样改变就诊。患者治疗以手术为主。其中3例行外阴单纯切除术,1例因一般情况较差及内科合并症,未行手术,给予放疗。1例患者行外阴广泛切除及腹股沟淋巴结清扫术,术后给予放疗。结论:外阴Paget病多见于绝经后患者,症状以瘙痒为主,有就诊延迟现象。治疗以手术为主,可选择外阴单纯切除术或局部病灶切除,伴有浸润性病变、合并腺癌或淋巴结转移者常需扩大手术范围,行外阴广泛切除。对不能手术的患者,可给予放疗。复发常见,患者均需终生随访。
Objective: To investigate the clinical features and treatment of Vulvar Paget’s disease. Methods: A retrospective analysis of 5 cases of vulvar Paget disease clinical data. Results: There were 5 cases of Paget disease in the vulva, aged 45-75 years, with an average of 67.4 years. 4 cases of menopause. In 5 cases, pruritus was the first symptom in 3 cases (60%) and in 2 cases (40%) due to vulvar nodules, lesions or eczema. Patients treated mainly surgery. 3 cases of vulvar simple excision, 1 case due to poor general condition and medical complications, no surgery, given radiotherapy. One patient underwent extensive excision of the vulva and dissection of the inguinal lymph nodes. Radiotherapy was given postoperatively. Conclusion: The genital Paget disease is more common in postmenopausal patients with pruritus and delayed treatment. Surgery-based treatment can choose vulvar excision or local lesion resection, associated with infiltrative lesions, with adenocarcinoma or lymph node metastasis often need to expand the scope of surgery, the vulva extensive excision. For patients who can not surgery, radiotherapy may be given. Common recurrence, patients are required lifetime follow-up.