软组织Rosai-Dorfman病1例及文献复习  

Rosai-Dorfman disease of soft tissue: A case report and literature review

作　　者：杨兆瑞[1] 路光中[1] 胡宏慧[1] 张宜玲[1] 

机构地区：[1]上海交通大学附属第一人民医院病理科,上海200080

出　　处：《第四军医大学学报》2006年第3期264-266,共3页Journal of the Fourth Military Medical University

摘　　要：目的探讨软组织罗-道病(RDD)的病理诊断和免疫组化特点以及鉴别诊断.方法通过HE、免疫组化及特殊染色观察1例下肢软组织RDD并复习文献.结果镜下可见丰富的胶原纤维和组织细胞增生以及大量浆细胞、淋巴细胞和嗜酸粒细胞浸润,组织细胞呈梭形、多边形,胞质嗜酸性颗粒状、放射条纹状或泡沫状,一些大组织细胞质内有多量小淋巴细胞.增生的胶原和梭形组织细胞构成不典型Storiform结构;组织细胞表达S-100,KP-1,MAC387,VIM,Masson三色及网状纤维染色显示大量胶原纤维增生,组织细胞PAS-AB染色阴性.结论软组织RDD是临床罕见的疾病,在病理诊断方面,易误诊为其他疾病,明确病理组织学特点在鉴别诊断中有重要意义.AIM： To study the pathological diagnosis, immunohistochemieal features and differential diagnosis of Rosai-Dorfman disease （RDD） of soft tissue. METHODS： A case of RDD of soft tissue on the lower limb was investigated by light microscopy, HE stain, silver stain, Masson stain and immunohistochemieal stains for S-100, KP-1, MAC387, VIM, UCHL-1, L26 and CD34, and related literatures were reviewed. RESULTS： Microscopically, the lesion consisted of abundant collagen fibrils, proliferated histioeytes and numerous infiltrated plasmocytes, lymphocytes and eosinophilic granulocytes. Fusiform or polygonal histiocytes had abundant, granular, eosinophilie cytoplasm or clear cytoplasm containing radially oriented strands or resembled xanthoma cells. Emperipolesis of numerous small lymphocytes was seen in the cytoplasm of some large histioeytes. Proliferated colla- gen fibrils and fusiform histioeytes formed atypical storiform pat- tern. Immunohistoehemieally, the proliferated histiocytes were positive for S-100, KP-1, MAC387 and VIM. Masson stain and silver stain revealed the abundant collagen fibrils in the lesion. The histiocytes were negative for PAS-AB. CONCLUSION： RDD of soft tissue is rare and liable to be misdiagnosed in pathologic diagnosis. The histopathologie features are therefore of significance in differential diagnosis.

关 键 词：Rosai-Doffman病 软组织 组织细胞 伸入运动 

分 类 号：R551.2[医药卫生—血液循环系统疾病]