乳腺外佩吉特病诊疗进展  

Advances in the diagnosis and treatment of extramammary Paget's disease

作　　者：雷攀 解晓都 张鹏[1] 余佳成 杨楠 梁培禾[1] LEI Pan;XIE Xiaodu;ZHANG Peng;YU Jiacheng;YANG Nan;LIANG Peihe(Department of Urology,Second Affiliated Hospital of Chongqing Medical University,Chongqing 400072,China)

机构地区：[1]重庆医科大学附属第二医院泌尿外科,重庆400072

出　　处：《西南医科大学学报》2024年第6期488-493,501,共7页Journal of Southwest Medical University

基　　金：重庆市自然科学基金项目(CSTB2023NSCQ-MSX0195);重庆市留学人员回国创业创新支持计划(cx2019146)。

摘　　要：乳腺外佩吉特病(extramammary Paget's disease,EMPD)是一种发现历史较长的罕见皮肤恶性肿瘤,好发于老年人,不同国家、地区发病情况存在明显差异,整体上随着人口老龄化的加剧,发病率呈上升趋势。本病生物学行为常表现为惰性,临床上易与多种皮肤疾病混淆,加之发病机制,尤其是细胞起源仍存有争议,常导致病程迁延,诊断被延误。EMPD可分为原发和继发两类,其诊断及评估强调活检,并应注意伴发身体内肿瘤的检查。EMPD的治疗复发率较高,可分为手术和非手术两类,前者关键点在于病灶切除范围的确定,术中/切缘病理检查较为重要;后者目前尚缺少统一规范的方案。本文就EMPD的诊疗及研究现状进行评述,以期为本病相关临床医生及研究人员提供参考。Extramammary Paget′s disease(EMPD)is a rare cutaneous malignancy with a long discovery history,predominantly affecting the elderly.The incidence of EMPD varies significantly across different countries and regions,and overall,it has been rising in parallel with the aging population.The biological behavior of EMPD is typically indolent,and it is often clinically confused with various dermatological conditions.Additionally,the pathogenesis,especially the cellular origin,remains controversial,frequently resulting in a prolonged course and delayed diagnosis.EMPD can be classified into primary and secondary types.Diagnosis and evaluation emphasize biopsy,with attention also required for screening for associated internal malignancies.EMPD has a high recurrence rate and treatment strategies can be broadly categorized into surgical and non-surgical approaches.The former focuses on determining the extent of lesion excision,with intraoperative margin pathology examinations being crucial.Standardized protocols are still lacking for the latter.This article reviewed the current state of diagnosis,treatment,and research of EMPD,aiming to provide a reference for clinicians and researchers involved in this condition.

关 键 词：乳腺外佩吉特病 皮肤恶性肿瘤 外阴 诊断 治疗 

分 类 号：R737.9[医药卫生—肿瘤]