累及骨和软骨Rosai–Dorfman病2例报告并文献复习

Rosai–Dorfman disease of bone and cartilage: two case reports and review of the literature

目的:探讨2例累及骨和软骨的结外罗道(Rosai–Dorfman)病的临床病理特征、诊断及鉴别诊断。方法:复习分别位于右胫骨近端及甲状软骨的2例Rosai–Dorfman病患者的临床和影像学资料,行组织学观察及免疫组织化学分析,并复习相关文献。结果:39岁女性,右胫骨占位及38岁男性,甲状软骨肿物。影像学示前者右胫骨上段溶骨性骨质破坏;CT示后者甲状软骨前实性占位,与甲状软骨界限不清。光镜下前者病变在破碎骨小梁间生长,后者病变包绕并侵犯甲状软骨,并在软骨化骨骨小梁间侵袭性生长。低倍镜下组织细胞显著增生,与浸润的淋巴细胞、浆细胞形成明暗相间的结构,部分组织细胞体积较大,呈多边形或椭圆形;胞浆淡嗜酸性或空亮,泡状核,可见小核仁;部分胞浆内见吞噬完整的淋巴细胞和(或)浆细胞、中性粒细胞等。免疫组织化学标记组织细胞表达S–100蛋白和CD68,不表达CD1a。结论:累及骨和软骨的Rosai–Dorfman病罕见,临床及影像学检查均容易误诊。组织学形态及免疫组织化学检查是确诊的唯一依据。

Objective： To investigate clinicopathologic features,diagnosis,and differential diagnosis of extranodal Rosai–Dorfman disease from bone and cartilage.Methods： Clinical data and morphological features of two cases of Rosai–Dorfman disease—located in the left tibia and thyroid cartilage—were retrospectively analyzed.Immunohistochemistry was employed and the related literature were reviewed.Results： A 39-year-old female and 38-year-old male had lesions in the left tibia and thyroid cartilage,respectively.X-ray images showed the former to be an oval lytic lesion,while CT showed the latter to be solid,occupying the area around the thyroid cartilage.Histopathologic features displayed diffuse infiltration of large histiocytes,1ymphocytes,plasma cells,and neutrophils that formed light and dark appearance under low power microscope.The large histiocytes had abundant cytoplasm,pale to eosinophilic in appearance,with a vesicular nucleus and a small basophilic nucleolus.Some histiocytes phagocytized intact lymphocytes or plasma cells（so-called emperipolesis）.Immunohistochemical staining showed histiocytes positive for S-100 protein and CD68,negative for CD1a.Conclusion： Rosai–Dorfman disease of bone and cartilage is extremely rare,and therefore easily misdiagnosed by clinicians and pathologists.Histopathologic characteristics and immunohistochemical findings are the main points for the diagnosis of this disease.