乳房外Paget病97例临床及组织病理分析

摘要

Objective Through the study of 97 cases of extramammary Paget's disease to analyze its clinical and pathological features. Methods A total of 97 cases of extramammary Paget's disease were treated in our department from September 2003 to December 2011, and their clinical manifestations, pathological features, treatment options and prognosis were retrospectively analyzed here. Results Both male and female could get extramammary Paget's disease, more male than female. Extramammary Paget's disease occurred mainly in apocrine sweat glands distributed rich region. It occurred mainly in the scrotum, penis, perineum, crissum, labia and monsveneris, and a small fraction occurred in the groin, armpits, chest and buttocks, which mostly occurs in scrotum. It should be identiifed with eczema, Bowen's disease and other diseases, clinically misdiagnosed. Typical pathological manifestations were scattered or lumps of Paget's cells in the epidermis. Immunohistochemistry stain showed that the expression of CEA, CDX2 and CAM5.2 (CK8/18) were positive, and negative for S-100 and EMA, this facilitated the distinguishing of the disease from the others. The expression of CK7 and CK20 was not consistent in different parts. Conclusion Early biopsy is helpful to reduce the misdiagnosis.%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。