乳房外Paget病68例临床及组织病理分析

Clinical and histopathological analysis of extramammary Paget’s disease in 68 patients

目的:总结乳房外Paget病(EMPD)的临床、组织病理学特点。方法:对2006年—2016年就诊于苏州大学附属第一医院的68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7.5∶1;平均发病年龄为(72.02±11.42)岁。单发性皮损62例占91.18%,皮损多累及生殖器部位,临床诊断与病理诊断符合率为86.76%。免疫组化细胞角蛋白(CK)7、癌胚抗原(CEA)表达阳性,CK5/6表达部位与正常对照组不同,CK20、黑色素瘤抗体(HMB)-45表达阴性。术后复发率为30%,12例复发病例中,10例肿瘤细胞累及附属器或真皮。结论:乳房外Paget病多见于老年男性,病程缓慢,组织病理提示浸润性生长的复发率高,免疫组化提示EMPD有腺体分化能力,本病术后复发率高,需长期临床随访。

Objective:To elucidate the clinical and histological features of extramammary Paget’s disease(EMPD).Methods:We retrospectively analyzed the clinical and pathological data of 68 patients from the First Affiliated Hospital of Soochow University from 2006 to 2016.Results:The male/female ratio was 7.5∶1.The average age of onset was 72.02±11.42 years old.Sixty two cases(91.18%)had single lesion,primarily located on the genital area.Consistency rate of clinical and pathological diagnosis was 86.76%.Immunohistochemistry showed positive for CK7 and CEA positive,and negative for CK20 and HMB-45.Location of CK5/6 expression in the skin differed between patients and normal controls.Postoperative recurrence rate was 30%.Among 12 recurrent cases,involvements in the appendages and the dermis were found in 10 cases.Conclusions:EMPD is more common in the elder males,with slow progression.Histopathology reveals invasive growth with higher recurrence rate.Immunohistochemistry suggests that glands in EMPD remain differentiation ability.Because of the high recurrence rate,this disease requires long-term clinical follow-up.