【摘要】 探讨原发性乳腺外Paget病的临床病理特征。方法回顾性统计分析49例原发性乳腺外Paget病的临床病理特征并行免疫组化染色。结果患者男性36例,女性13例,年龄47~93岁,平均68.3岁。临床主要表现为皮肤湿疹样改变或新生物,并有瘙痒、疼痛等表现,多累及生殖器或肛周等会阴部位。大体见皮损呈湿疹样红斑、融合性丘疹并可伴渗液、糜烂等改变。镜下表皮全层内见数目不等的Paget细胞浸润,呈单个散在、巢团状或假腺样分布,部分病例可见病变累及皮肤附属器,小部分病例可见肿瘤浸润真皮;Paget细胞呈圆形或卵圆形上皮样,胞质丰富,淡染或透明,核大有异型。免疫组化:49例CK7均(+),部分p16(+),CK5/6和p63(-)。32例获得随访资料,其中复发8例。结论乳腺外Paget病发病率低,好发于老年男性会阴部,临床需与良性皮肤病鉴别。Paget细胞具有特殊的组织形态特点,免疫表型可以帮助鉴别诊断其他多种表皮内原位肿瘤。更多还原

【Abstract】 Objective To study the clinicopathological characteristics of extramammary Paget’s disease( EMPD).Methods The routine HE,immunohistochemical staining and clinicopathological features were analyzed in 49 cases of EMPD with review of the literature. Results 36 cases were male and 13 patients were female. Patients aged 47 to 93 years,with average 68. 3 years. Clinical findings were eczematoid lesions or neoplasm with significant pruritus and pain,skin lesions commonly involving the genitals,vulvar or anus,etc. Gross pathologic examination of EMPD revealed eczema,erythema or confluent papules with exudates and erosion. Histologically,EMPD was characterized by the presence of large oval or polyhedral intraepithelial Paget cells that had pale cytoplasm and large nuclei with prominent nucleoli. Paget cells were arranged either singly or in clusters throughout the epithelium, and sometimes formed gland-like structures.Immunohistochemically,Paget cells expressed CK7 and p16 in partial cases,p63 and CK5/6 were negitive. Follow-up showed 8 recurrence in 32 cases. Conclusions EMPD is a rare tumor with a male preponderance especially in perineum which has special histological features. Immunohistochemical stain can help for differential diagnosis from a variety of other skin carcinoma in situ.更多还原