摘要
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.
Objective To evaluate the clinicopathological features of extramammary Paget＆#39;s disease.Methods Statistical analyses were performed for the clinicopathological features for 75 cases of extramammary Paget＆#39;s disease from 2004 to 2013.Results Extramammary Paget＆#39;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＆#39;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＆#39;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.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2015年第22期1751-1754,共4页
National Medical Journal of China
基金
北京大学医学联合研究种子基金（2014-MB-25）的支持
关键词
PAGET病
乳房外
诊断
治疗
预后
Paget＇s disease, extramammary
Diagnosis
Therapy
Prognosis
