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目的通过对97例乳房外Paget病患者临床资料回顾性研究,分析其皮损部位及组织病理及免疫组化特点。方法收集我科2003年9月—2011年12月收治的97例乳房外Paget病患者临床资料,对其皮损部位及组织病理特点进行回顾性分析。结果乳房外Paget病男女均可发病,男性发病多于女性,平均发病年龄为63岁。皮损主要发生于顶泌汗腺分布较丰富的区域,如阴囊、阴茎、会阴、肛周、阴唇、阴阜等部位,少部分发生于腹股沟、腋窝、前胸、臀部。本病临床上易误诊。典型的组织病理表现为表皮内散在或成团块状分布的Paget细胞。免疫组化染色显示CEA、CDX2、CAM5.2(CK8/18)阳性,S-100蛋白、EMA等阴性,有利于鉴别,而CK7和CK20在不同部位的表达总分不一致。结论对乳房外Paget病早期行组织病理检查有利于减少临床误诊。
Objective To retrospectively study the clinical data of 97 cases of extramammary Paget’s disease and analyze the histological and immunohistochemical features of the lesions. Methods The clinical data of 97 patients with extramammary Paget disease who were treated in our department from September 2003 to December 2011 were collected and their lesion sites and histopathological features were retrospectively analyzed. Results Out-of-breast Paget disease can occur in both males and females, with more males than females, with an average age of onset of 63 years. Skin lesions occur mainly in the more abundant areas of the apocrine sweat glands, such as the scrotum, penis, perineum, perianal crease, labia minora, and other parts of the mons pubis, a small part of the groin, armpits, chest, buttocks. The disease is clinically misdiagnosed. Typical histopathology is Paget cells scattered in interstitial or lumpy clumps. Immunohistochemical staining showed that CEA, CDX2, CAM5.2 (CK8 / 18) positive, S-100 protein, EMA and other negative, is conducive to the identification, and CK7 and CK20 expression in different parts of the total score is inconsistent. Conclusion The early stage of extramammary Paget disease histopathological examination is conducive to reduce the clinical misdiagnosis.