外阴派杰病12例临床分析

Clinical Analysis of 12 Cases of Vulvar Paget＆#39;s Disease

【目的】探讨外阴派杰病（Paget＇s disease）的临床特点、治疗方式及其对预后的影响。【方法】回顾分析12例外阴派杰病的临床资料，观察和分析其临床特点及治疗方式对预后的影响。【结果】12例患者的中位数发病年龄为62岁。出现症状或发现外阴病变到组织学确诊中位数时间为29个月。双侧外阴受累多见，共8例（66．7％）。12例患者中合并外阴癌1例，1例随访发现宫颈原位癌，1例3年后发现乳腺癌。12例患者接受了手术治疗：单纯手术治疗9例，其中5例复发，另1例复发患者因2次复发而行放射治疗，这6例复发患者中病理提示切缘受累共5例；联合治疗3例，无一例复发。在12例患者中共有2例进行了腹股沟淋巴结清扫，其中1例因合并外阴癌行此术，另1例因复发，行腹股沟淋巴结清扫后仍出现了再次的复发。【结论】外阴派杰病多见于绝经后妇女，临床诊断延迟现象明显。病变多累及双侧外阴，切缘受累现象常见。常合并相关的内在肿瘤是其又一特点。手术是最常用的治疗方式，术后复发率较高与切缘受累明显相关。淋巴结清扫不作为常规进行。联合的治疗方式可能更有益于预后。

[Objective]To explore clinical characteristics and treatment method of vulvar Paget＆#39;s disease and the impact on the prognosis. [Methods] Clinical data of 12 patients with vulvar Paget＆#39;s disease were analyzed retro‐spectively. The impact of clinical characteristics and treatment method on the prognosis were observed and ana‐lyzed. [Results]The median age of 12 patients was 62 years old. The median time between initial symptom or vul‐var lesion and definite pathological diagnosis was 29 months. Both sides of 8 patients（66. 7% ） were involved. A‐mong 12 patients, 1 patient was complicated with vulvar cancer, and 1 patient had cervical carcinoma in situ during the follow up, and 1 patient had breast cancer after 3 years. The 12 patients underwent the operation. Among the 12 patients, 5 patients of 9 patients undergoing simple operation had the recurrence. One recurrent patient received radiotherapy due to the second recurrence. Among 6 recurrent patients, the involvement of incision margin of 5 pa‐tients was confirmed by pathology. None of 3 patients undergoing the combination therapy recurred. Inguinal lymph node dissection was performed in 2 patients of 12 patients due to vulvar cancer and recurrence. After ingui‐nal lymph node dissection, the recurrence occurred again. [Conclusion]Vulvar Paget＆#39;s disease is commonly seen in women after menopause. Clinical diagnosis is usually delayed. Both sides of vulva are often involved. It is common to find the involvement of incision margins. Moreover, the other feature is the complication with internal malig‐nancies. Surgery is the common treatment method. High postoperative recurrent rate is obviously related with the involvement of incision margins. Lymph node dissection is not taken as the routine method. The combination ther‐apy may be better for the prognosis.