原发性外阴Paget病36例临床分析  

Analysis of clinical characters of female vulvar Paget disease

作　　者：张晨昱 孙智晶[1] 朱兰[1] 郎景和[1] 成宁海[1] 王文泽[2] Zhang Chenyu;Sun Zhijing;Zhu Lan;Lang Jinghe;Cheng Ninghai;Wang Wenze(Department of Obstetrics and Gynecology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医院妇产科,100730 [2]中国医学科学院北京协和医院病理科,100730

出　　处：《中华妇产科杂志》2018年第8期540-546,共7页Chinese Journal of Obstetrics and Gynecology

摘　　要：目的 探讨原发性外阴Paget病（VPD）的临床病理特点和诊治情况，进一步分析影响原发性VPD患者复发的相关因素。方法收集1983年1月-2017年12月中国医学科学院北京协和医院收治的经病理检查证实为原发性VPD的患者共36例，回顾性分析其临床病理特点、诊断、治疗、预后，并分析影响原发性VPD患者复发的相关因素。结果（1）临床病理特点：原发性VPD好发于绝经后妇女，占94％（34／36）；主要症状为外阴瘙痒，占86％（31／36）；主要体征为外阴皮损，常表现为溃疡（67％，24／36）、红斑（50％，18／36）、色素减退（42％，15／36）、硬结（31％，11／36）、色素沉着（17％，6／36），病变主要累及大阴唇（97％，35／36）、小阴唇（53％，19／36）、阴蒂（28％，10／36）、肛周（25％，9／36）、阴道口（3％，1／36）、尿道口（3％，1／36）。19％（7／36）的患者合并外阴或其他部位的上皮内瘤变或原发性恶性肿瘤。（2）诊断和治疗：36例患者均经病理检查确诊为原发性VPD。原发性VPD的治疗以手术治疗为主。36例原发性VPD患者中，34例（94％，34／36）患者行手术治疗，2例（6％，2／36）患者为非手术治疗。手术治疗方式包括病灶切除术、病灶扩大切除术、单纯外阴切除术、外阴广泛性切除术；非手术治疗方式包括放疗、化疗、激光治疗、光动力治疗等。（3）预后：36例原发性VPD患者中，4例失访，随访率为89％（32／36）。随访时间为术后1个月-31年，中位随访时间35．3个月。34例手术治疗的原发性VPD患者中30例有随访资料，其中2例患者随访时间未达到6个月无法判断是否复发，8例患者手术失败（即术后半年内症状再次出现，且经病理检查证实有Paget细胞），可评价是否复发的患者20例，其中9例复发，复发率为45％（9／20），中位复发时间为术后14个月。Objective To investigate the clinical and pathological features, diagnosis and treatment of primary vulvar Paget disease （VPD）, and analyze the related factors that may affect the recurrence. Methods A retrospective study was carried out on 36 patients diagnosed as VPD pathologically from January 1983 to December 2017 at Peking Union Medical College Hospital, Chinese Academy of Medical Sciences. The clinical and pathological features, diagnosis, treatment and prognosis and the factors influencing recurrence rate of VPD were analyzed. Results （1） Totally 94% （34/36） of VPD occurred in postmenopausal women. Pruritus was counted 86% （31/36） of the main complaint. Lesions of vulvar were main symptom which had no specificity, acting as ulcer （67%, 24/36）, erythema （50%, 18/36）, depigmentation （42%, 15/36）, sclerosis （31%, 11/36）, and pigmentation （17%, 6/36）. The lesions invaded labium majus （97%, 35/36）, sometimes labium minus （53%, 19/36）, clitoris （28%, 10/36）, perianal （25%,9/36）, orificium vaginae （3%, 1/36）, and meatus urinarius （3%, 1/36）. Approximately 19%（7/36） of VPD coexisted with intraepithelial neoplasia or adenocarcinoma of vulvar or other part of body. （2） Diagnosis and treatment： diagnosis was confirmed histologically by biopsy or pathologies after surgery, and immunohisto- chemical results were helpful for differential diagnosis. Surgery was the mean treatment method, 34 of all the 36 patients （94%, 34/36） underwent surgery for at least once, while 2 patients （6%, 2/36） were performed non-operative treatment. The surgical treatment included excision of focus, wide local excision, simple vulvectomy, and extensive vulvectomy. The non-operative treatment included radiotherapy, chemotherapy, laser, photodynamic therapy, and so on. （3）Prognosis： among 36 VPD patients, 4 were lost to follow-up with a 89% （32/36） follow-up rate. Median follow-up was 35.3 months （range, 1 month to 31 years）. During the follow-up period

关 键 词：外阴肿瘤 佩吉特病 乳腺外 预后 

分 类 号：R737.35[医药卫生—肿瘤]