原发性乳房外Paget病43例临床病理分析

Clinical and Histopathological Analysis of 43 Patients with Primary Extramammary Paget′s Disease

目的探讨原发性乳房外Paget病的临床表现与组织病理特征。方法回顾分析43例原发性乳房外Paget病患者的临床和病理资料。结果43例患者中,男41例,女2例,年龄49~101岁,病程1个月~15年。皮损主要位于阴囊、阴茎、阴阜等外生殖器部位(41/43例,95.35%),其次为腋下(2/43例,4.65%)。临床诊断与病理检查的符合率为46.51%(20/43例)。组织病理结果显示33例(76.74%)为原位乳房外Paget病,10例(23.26%)为侵袭性乳房外Paget病,35例(81.40%)侵犯附属器,17例(39.53%)出现棘层松解现象。Paget细胞侵袭深度与患者病程相关(P=0.010),未发现与确诊年龄(P=0.081)、肿瘤面积(P=0.707)、附属器受累(P=0.101)、棘层松解现象(P=0.656)有关。侵袭性乳房外Paget病中,Paget细胞除在真皮内形成大小不等的团块外,还可形成腺样和结节状结构。39例行局部扩切手术,3例行Mohs显微描记手术,1例行光动力术治疗。术后随访的35例患者中8例复发,复发率22.86%,均为行局部扩切术的患者。结论原发性乳房外Paget病在我国多见于老年男性,临床表现易与股癣、湿疹等相混淆,Mohs显微描记手术已成为该病的首选治疗方法,可有效降低复发率。

Objective To elucidate the clinical manifestations and histopathological characteristics of primary extramammary Paget′s disease(EMPD).Methods Clinical data and biopsy specimens were collected from 43 patients with primary EMPD and analyzed retrospectively.Results Among the 43 patients,41 were males and two were females.The age of onset ranged from 49 to 101 years.The duration of disease varied from one month to 15 years.Skin lesions most frequently occurred on the genital area such as scrotum,penis and vulva(41/43,95.35%),followed by the armpit(2/43,4.65%).The concordance between clinical and pathological diagnoses was 46.51%(20/43).Histopathological results showed that 33 cases(76.74%)were in situ and 10 cases(23.26%)were invasive.Adnexal involvement in primary EMPD was a very common feature(35/43,81.40%)and acantholysis phenomenon in the epidermis could also be seen(17/43,39.53%).Comparing Paget cells located in dermis and those confined to epidermis,significant differences existed in course(P=0.010),but not in age of onset(P=0.081),lesion size(P=0.707),adnexal involvement(P=0.101)and acantholysis phenomenon(P=0.656).Paget cells in invasive EMPD usually located in dermis forming nest-like patterns,as well as glandular and nodular structures.Thirty-nine patients underwent local resection,three patients Mohs micrographic surgery and one patient photodynamic therapy.Eight of the 35 patients followed-up had recurrence,all of whom underwent local resection.Conclusion Primary EMPD is more common among elder males in China.Clinically,their symptoms and morphology mimicked dermatoses such as tinea cruris and eczema.Mohs surgery can effectively reduce recurrence rate.