乳房外Paget病135例临床及组织病理学特征分析  

Analysis on the Clinical and Histopathological Characteristics of 135 Patients with Extramammary Paget′s Disease

作　　者：朱一维 张恋 顾洪芝 王惠芬 罗娜 王娟 翟志芳 ZHU Yiwei;ZHANG Lian;GU Hongzhi;WANG Huifen;LUO Na;WANG Juan;ZHAI Zhifang(Department of Dermatology,the First Affiliated Hospital of Army Medical University,Chongqing 400038,China;Department of Dermatology,the Third Affiliated Hospital of Chongqing Medical University,Chongqing 401120,China)

机构地区：[1]陆军军医大学第一附属医院皮肤科,重庆400038 [2]重庆医科大学附属第三医院皮肤科,重庆401120

出　　处：《中国皮肤性病学杂志》2024年第2期171-176,共6页The Chinese Journal of Dermatovenereology

基　　金：重庆市技术创新与应用发展专项重点项目(CSTB2022TIAD-KPX0181)。

摘　　要：目的总结并分析乳房外Paget病(extramammary Paget′s disease,EMPD)的临床及组织病理学特征,为临床诊疗提供参考。方法收集2003年9月—2023年2月就诊于本科,经临床及组织病理检查确诊的EMPD患者的临床及组织病理学资料,进行回顾性总结分析。结果共纳入EMPD患者135例,平均就诊年龄(67.3±10.2)岁,男女比例为4.6∶1,病程1个月~30年;常见受累部位男性依次为阴囊、阴茎、阴阜区及肛周,女性依次为阴唇及肛周;就诊时以红斑、斑块、丘疹结节为常见皮损;首诊误诊率达56.30%。组织病理主要表现为表皮内Paget细胞散在或呈巢状分布,75例发生真皮浸润及皮肤附属器受累,48例有肿瘤细胞间棘层松解现象。复发转移组组织病理出现真皮、附属器浸润患者数明显高于无复发转移组,两组间差异有统计学意义(P<0.05)。多因素COX比例风险模型显示,患者年龄及组织病理上是否发生棘层松解和/或真皮、附属器浸润是肿瘤复发转移的危险因素。结论EMPD好发于老年人,男性高于女性,临床误诊率高。发病年龄高、组织学上出现棘层松解、肿瘤真皮浸润及附属器受累与肿瘤的复发、转移及更差的预后相关。Objective To analyze the clinical and histopathological characteristics of patients with extramammary Paget′s disease(EMPD)and provide references for clinicians.Methods The clinical and histopathological data of 135 patients with EMPD were retrospectively analyzed,who visited our department from September 2003 to February 2023 and were diagnosed as EMPD by by clinical and histopathological examination.Results A total of 135 patients with EMPD were enrolled,with the average age of(67.3±10.2)years old and a variable disease course from 1 month to 30 years.The male/female ratio was 4.6∶1.In turn,the lesions mainly located on the scrotum,penis,mons pubis and perianal in male patients and on the labia and perianal in female patients.The lesions manifested successively as erythema,plaques,papules and nodules,and so on.The misdiagnosis rate was up to 56.30%at the first visit.The typical histopathology showed Paget cells were distributed within the epidermis in the scattered or nested pattern.Dermal infiltration and adnexal involvement were observed in 75 cases and acantholysis phenomenon in the epidermis and/or dermis occurred in 48 cases.The cases with dermal and adnexal infiltration in the recurrence and metastasis group was significantly more than that in the none one(P<0.05).The Cox′s proportional hazards regression model showed that both the elder and with infiltration of dermis and appendages and/or acantholysis were the risk factors for recurrence and metastasis of EMPD.Conclusion EMPD was more common in the male elder,with high misdiagnosis rate.The elder age of onset,acantholysis phenomenon,dermal infiltration and adnexal involvement were associated with recurrence,metastasis and worse prognosis.

关 键 词：PAGET病 乳房外 Paget细胞 临床特征 组织病理 

分 类 号：R739.5[医药卫生—肿瘤]