CAJ | 학술논문

目的总结乳房外Paget病(EMPD)的临床病理特点.方法对2004至2013年就诊于北京大学第一医院的75例EMPD患者的临床、病理资料进行回顾性统计分析.结果患者男女比例为5.25∶1;平均发病年龄为(64.23 ±12.02)岁.皮损单发者占92.0％(69/75),多累及生殖器部位;61.6％ (45/73)的患者有瘙痒症状.侵袭性EMPD占全部患者的22.7％(17/75).该病临床诊断与病理诊断符合率为85.3％ (64/75),11例误诊病例中7例误诊为湿疹.肿瘤细胞累及附属器上皮或真皮的患者与肿瘤细胞局限于表皮内的患者比较,发病年龄、病程时间和复发率差异有统计学意义(均P ＜0.05),皮损大小差异无统计学意义.67.1％ (47/70)的患者行Mohs手术治疗,其中38.3％(18/47)的患者1次切净,61.7％ (29/47)需2次以上切除.经统计推测该病术后5年复发率高达25.0％;Mohs术后5年的复发率为18.0％,非Mohs术后5年的复发率为36.5％.7例复发病例中,3例为2011至2013年期间术后未行放射治疗的患者,同期15例行放射治疗的患者均未复发.结论中国人群EMPD常见于老年男性,病程缓慢,其侵袭程度与复发率相关,故早期诊断非常重要.本病肉眼范围与病理细胞学边界差异较大,Mohs手术有助于切除边界的掌控,同时,合并放疗能降低复发率.
목적 총결유방외Paget병(EMPD)적림상병리특점.방법 대2004지2013년취진우북경대학제일의원적75례EMPD환자적림상、병리자료진행회고성통계분석.결과 환자남녀비례위5.25∶1;평균발병년령위(64.23 ±12.02)세.피손단발자점92.0％(69/75),다루급생식기부위;61.6％ (45/73)적환자유소양증상.침습성EMPD점전부환자적22.7％(17/75).해병림상진단여병리진단부합솔위85.3％ (64/75),11례오진병례중7례오진위습진.종류세포루급부속기상피혹진피적환자여종류세포국한우표피내적환자비교,발병년령、병정시간화복발솔차이유통계학의의(균P ＜0.05),피손대소차이무통계학의의.67.1％ (47/70)적환자행Mohs수술치료,기중38.3％(18/47)적환자1차절정,61.7％ (29/47)수2차이상절제.경통계추측해병술후5년복발솔고체25.0％;Mohs술후5년적복발솔위18.0％,비Mohs술후5년적복발솔위36.5％.7례복발병례중,3례위2011지2013년기간술후미행방사치료적환자,동기15례행방사치료적환자균미복발.결론 중국인군EMPD상견우노년남성,병정완만,기침습정도여복발솔상관,고조기진단비상중요.본병육안범위여병리세포학변계차이교대,Mohs수술유조우절제변계적장공,동시,합병방료능강저복발솔.
Objective To evaluate the clinicopathological features of extramammary Paget's disease.Methods Statistical analyses were performed for the clinicopathological features for 75 cases of extramammary Paget's disease from 2004 to 2013.Results Extramammary Paget's disease exhibited a male predominance (5.25∶1).The mean age of onset was (64.23 ± 12.02)years.Single lesion accounted for 92.0％(69/75) and most of them were located in genital area.Pruritus occurred in 61.6％ (45/73) patients.Invasive extramammary Paget's disease accounted for 22.7％ (17/75).The concordance between clinical and pathological diagnoses was 85.3％ (64/75).Among 11 misdiagnosed cases,7 cases were misdiagnosed as eczema.Comparing Paget cells located in epithelium of adnexa or dermis and those confined to epidermis,significant differences existed in age of onset,coume,recurrence rate(all P ＜ 0.05),but not in lesion size.Among 67.1％ (47/70) of patients undergoing Mohs surgery,38.3％ (18/47) had a complete one-time resection while 61.7％ (29/47) required more than twice for thorough resection.The 5-year recurrence rate was 25.0％.The postoperative 5-year recurrence rate of Mohs micrographic surgery (MMS) was 18.0％ and the rate of non-MMS 36.5％.From 2011 to 2013,3 recurrent cases had no radiotherapy.However another 15 cases on radiotherapy were non-recurrent.Conclusions With a slow progression,extramammary Paget's disease is more common among elder males.Recurrence rate is associated with the degree of invasion.Thus an early diagnosis is essential.Due to obvious differences between gross and pathological boundaries,Mohs surgery may help to determine the margin.Meanwhile,adding radiotherapy reduces the recurrence rate.