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目的研究外阴Paget病的临床病理特征、诊治和预后。方法回顾性分析和总结2000~2012年天津市中心妇产科医院收治的5例外阴Paget病的临床资料。结果5例外阴Paget病患者平均年龄53.6岁(48~57岁),外阴瘙痒和病灶是主要症状,从最初症状到确诊平均时间6.0年。所有患者初治均为手术治疗,均未行放化疗。1例行外阴单纯切除术,4例行外阴根治术。术后病理:2例为上皮内Paget病,2例为浸润性Paget病,1例合并腺癌。术后复发1例(合并腺癌)距初治时间10年,行二次手术。平均随访时间6.6年。结论本病好发于绝经后妇女,病情发展缓慢,普遍存在就诊延迟现象。病变多呈湿疹样,易被误诊。确诊依靠病理活检。治疗以手术切除为主。外阴根治术是主要手术方式。预后主要取决于病理类型。
Objective To study the clinicopathological features, diagnosis and prognosis of vulvar Paget’s disease. Methods The clinical data of 5 cases of genital Paget disease admitted to Tianjin Central Obstetrics and Gynecology Hospital from 2000 to 2012 were retrospectively analyzed and summarized. Results The average age of Vulvar Paget’s disease was 53.6 years (48-57 years) in 5 cases. Pruritus and lesions of the genital area were the main symptoms. The average time from initial symptom to diagnosis was 6.0 years. All patients were surgical treatment of initial treatment, no chemotherapy. 1 cases of vulvar simple excision, 4 cases of radical cure. Postoperative pathology: Paget’s disease in 2 cases, Paget’s disease in 2 cases and adenocarcinoma in 1 case. Postoperative recurrence in 1 case (with adenocarcinoma) from the initial treatment time of 10 years, the second operation. The average follow-up time of 6.6 years. Conclusion The disease occurs in postmenopausal women, the disease develops slowly, the prevalence of delayed treatment. Mostly eczematous lesions, easily misdiagnosed. Diagnosis depends on biopsy. Surgical treatment of the main. Vaginal radical surgery is the main surgical approach. The prognosis depends mainly on the type of pathology.