结外Rosai-Dorfman病临床病理特征分析  

Extranodal Rosai-Dorfman disease: a clinicopathologic analysis of sixteen cases

作　　者：冯帅 刘冲[2] 张智弘[2] 范钦和[2] 赵华 李海[2] FENG Shuai;LIU Chong;ZHANG Zhi-hong;FAN Qin-he;ZHAO Hua;LI Hai(Department of Pathology,Wuxi Maternal and Child Health Hospital Affiliated to Nanjing Medical University,Wuxi 214002,China;Department of Pathology,the First Affiliated Hospital of Nanjing Medical University,Nanjing 210029,China)

机构地区：[1]南京医科大学附属无锡妇幼保健院病理科,无锡214002 [2]南京医科大学第一附属医院病理科,南京210029

出　　处：《临床与实验病理学杂志》2019年第11期1325-1329,共5页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨结外Rosai-Dorfman病(Rosai-Dorfman disease,RDD)的临床病理学特征、诊断及鉴别诊断、治疗及预后。方法回顾性分析南京医科大学第一附属医院2014~2019年收治的16例结外RDD的临床和病理资料,并复习相关文献。结果16例结外RDD中,男性10例,女性6例,年龄12~69岁,平均年龄46.6岁。发病部位:鼻腔鼻窦4例、乳腺2例、腰部及前臂皮肤2例、骨2例,腮腺、肩部皮下、颞部软组织、眼球巩膜、颊黏膜、额部脑膜各1例。镜检:可见大组织细胞、淋巴细胞和浆细胞弥漫片状分布,低倍镜下呈典型的明暗相间结构,大组织细胞内可见伸入现象,部分病例间质内可见显著的纤维结缔组织反应及席纹状排列结构。免疫表型:组织细胞中S-100、CD68和CD163均阳性,CKpan、CK8/18、CD1a、Langerin、CD30和ALK均阴性,Ki-67增殖指数为1%~10%;背景淋巴浆细胞表达各自相应标志物:CD3、CD20、CD45、CD38和CD138均阳性。8例患者获得随访资料,随访6~58个月,肿块切除后均未复发。结论结外RDD相对罕见,临床表现不具特异性,部分病例具有非典型组织学形态,易误诊。熟悉并掌握其较为广谱的临床病理学谱系并结合S-100蛋白染色可将其与其它类似病变相鉴别。Purpose To investigate the clinicpathologic characteristics,differential diagnosis,treatment and prognosis of extranodal Rosai-Dorfman disease(RDD).Method The clinical and pathological data of 16 cases of extranodal RDD admitted to the First Affiliated Hospital of Nanjing Medical University from 2014 to 2019 were analyzed retrospectively,and the relevant literature was reviewed.Results The patients included ten males and six female.The age of patients were 12 to 69 with a mean age of 46.6 years.The sites of involvement were nasal cavity,mammary gland,skin of waist and forearm,bone,parotid gland,subcutaneous tissue of shoulder,temporal region,sclera and frontal meninges.Microscopically,extranodal RDD was characterized by cluster or sheets of large polygonal histiocytes intermingled with a florid lymphocyte and plasma cell infiltrate.The characteristic sharply alternating light and dark zones on low magnification and empiripolesis phenomenon seen in RDD histiocytes on high magnification were diagnostic useful.Some cases were remarkable for a prominent sclerotic fibrous stromal response and vague storiform pattern.Immunohistochemical study showed that RDD histiocytes cells showed strong diffuse staining for S-100 protein,CD68 and CD163.CKpan,CK8/18,CD1 a,Langerin,CD30 and ALK were negative in all tested cases.The Ki-67-labeling index was low.Lymphocyte and plasma cells were positive CD3,CD20,CD45,CD38 and CD138.Eight cases were followed up for 6 to 58 months,no cases relapse after resection.Conclusion Extranodal RDD is a rare lesion without characteristic clinical manifestations and it is easily misdiagnosed as other type of lesions.Recognition of its wide spectrum of clinical and histologic features combined with S-100 protein immunohistochemical staining is important to avoid confusion with other lesions.

关 键 词：ROSAI-DORFMAN病 伸入现象 诊断 鉴别诊断 

分 类 号：R593[医药卫生—内科学]