多发性皮下Rosai-Dorfman病1例报告并文献复习

目的:探讨多发性皮下Rosai-Dorfman病的临床病理特征、诊断、鉴别诊断。方法:对1例多发性皮下Rosai-Dorfman病进行组织形态学、免疫组化及特殊染色检查,并随访及复习相关文献。结果:肿块分别位于左侧胸壁及右腹股沟区,两处病变组织学上表现相似,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、胞浆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";细胞密集区散在淋巴滤泡伴较多浆细胞、中性粒细胞等浸润。免疫表型:组织细胞呈S-100、CD163及CD68阳性。结论:多发性皮下Rosai-Dorfman病罕见,确诊主要依靠形态学及免疫组化标记,无论临床及病理均需与纤维组织细胞瘤、Langerhans细胞组织细胞增生症、非特异性肉芽肿、炎性肌纤维母细胞性肿瘤等相鉴别,治疗以手术切除为主,预后较好。

Multiple subcutaneous rosai-dorfman disease: A case report and review of the literature

Objective:To investigate the clinicopathologic features,diagnosis,differential diagnosis of multiple subcutaneous Rosai-Dorfman disease(MSRDD).Methods: One case of multiple subcutaneous Rosai-Dorfman disease was treated in our hospital.We analyzed diagnosis,treatment,histopathology,immunohistochemistrical,and relative literature were reviewed.Results: The lesions were located in the left chest wall and right groin area.Histologically,two lesions were similar with nodular structures presenting with alternating hyper-and hypo-cellular areas.There were areas of hypo-cellular revealed lymph-sinustoid structure characterized by variable numbers of large histiocytes,which had an abundant cytoplasm,pale to eosinophilic in appearance,phagocytosed intact lymphocytes or emperipolesis.While hyper-cellular areas revealed lymphatic folliculus,infiltration of lymphocytes,plasma cells,neutrophils and numerous collagen fiber.Immunohistochemistrical profiles: the large histiocytes were strongly positive for S-100,CD163 and CD68 protein.Conclusion: Multiple subcutaneous Rosai-Dorfman disease is rare with benign behavior.In clinic and pathology,it need to differentiate diagnosis with fibrohistiocytoma,Langerhans cell histiocytosis,nonspecific granuloma,inflammatory myofibroblastic tumor,and so on.The primary approach of treatment for MSRDD is complete surgical excision and its prognosis is good.