摘要目的 探讨多发性Rosai-Dorfman病(Rosai-Dorfman disease,RDD)的临床病理特征、诊断、鉴别诊断及预后.方法 对7例多发性RDD进行组织形态学、免疫组织化学及特殊染色检查,并随访及复习相关文献,进行总结分析.结果 7例病例中,女性3例,男性4例,年龄18 ～ 60岁(中位年龄45.7岁),肿块最大径0.8～6.0 cm(平均为3.0 cm).肿块分别位于:左手臂皮下、甲状腺、气管旁;左上颌皮下、右颊皮下;左胸壁皮下、右腹股沟皮下;双侧颈部皮下;有胫骨上段、右大腿皮肤;右额叶、大脑廉旁;双侧颈部淋巴结.其中例1～6均为淋巴结外组织,而例7为淋巴结.7例均表现为渐进性增大的肿块.病理组织学上,7例表现相似,呈细胞疏松区和密集区交替分布的结节状结构,细胞疏松区可见淋巴窦样结构,其间有一些体积较大、胞质丰富淡染的组织细胞,可见胞质内吞噬物和淋巴细胞“伸入运动”;细胞密集区可见较多淋巴细胞浆细胞、中性粒细胞等浸润.其中2例中可见散在淋巴滤泡浸润.免疫表型:组织细胞呈S-100蛋白、CD163及CD68阳性.结论 多发性RDD罕见,确诊主要依靠形态学及免疫组织化学标记,临床及病理均需与肉芽肿性疾病、IgG4相关的硬化性疾病、炎性肌纤维母细胞瘤、纤维组织细胞瘤、Langerhans细胞增生症等相鉴别,治疗以手术切除为主,预后较好.

abstractsObjective To investigate the clinicopathologic features,diagnosis,differential diagnosis of multiple Rosai-Dorfman disease (RDD).Methods Seven cases of multiple RDD were treated in Fujian Provincal Hospital and Fuzhou General Hospital of Nanjing Military Command of Chinese PLA.The disease was analyzed,focusing on the process of diagnosis,the treatment and follow-up.Histopathology,immunohistochemical profiles and relative literature were reviewed to reveal the characteristics of this disease.Results The seven cases,occurred in 3 women and 4 men from 18 to 60 years of age (median 45.71 years),with masses measured of 0.8-6.0 cm (average size of 3.0 cm).Masses located in,left subcutaneous arm,thyroid,paratrachea,left maxilla,right subcutaneous cheek,left subcutaneous chest wall,right subcutaneous inguina,bilateral subcutaneous neck,right tibia,right thigh skin,right frontal lobe of brain,cerebral parafalx and bilateral lymph nodes of the neck,respectively.Among the cases,from the first case to the sixth case were extranodal tissue,and the seventh case was located in lymph nodes.Cases showed progressive increase of the mass.Histologically,all lesions of seven cases were similar with nodular structures presenting with alternating hyper-and hypo-cellular areas.The hypo-cellular areas revealed lymphsinustoid structure characterized by variable numbers of large histiocytes,which had an abundant cytoplasm,pale to eosinophilic in appearance,phagocytozed intact lymphocytes or emperipolesis.While hyper-cellular areas revealed the infiltration of lymphocytes,plasma cells,neutrophils and numerous collagen fiber.Two cases also revealed the infiltration of lymphoid follicles.Immunohistochemically,the large histiocytes were strongly positive for S-100,CD163 and CD68 protein.Conclusions Multiple RDD is rare.In clinic and pathology,it needs to be differentiated from granulomatous diseases,IgG4-related sclerotic diseases,inflammatory myofibroblastic tumor,fibrohistiocytoma,Langerhans cell histiocytosis,and so on.The primary approach of treatment for multiple RDD is complete surgical excision and its prognosis is good.