喉结外Rosai-Dorfman病临床病理观察

Extranodal Rosai-Dorfman disease:a case report and review of literature

目的探讨Rosai-Dorfman病的临床病理特征。方法对1例术前影像学诊断为喉恶性肿瘤病例,复习临床病史和影像学资料,行组织学检查、特殊染色(PAS、Giemsa和Warthin-Starryy银染色)和免疫组化标记(S-100、CD68、lyzosyme、SMA、p63、CD30、CD1a和desmin)并复习文献。结果患者为老年女性。CT示喉咽腔左1/2部软组织增厚影,局部可见隆起,病变累及左侧声门上区、厌前间隙、左侧真声带及前联合,直至环状软骨下约6.5 mm。组织学显示喉黏膜及黏膜下层大量浆细胞、淋巴细胞及组织细胞弥漫浸润,且累及周围横纹肌组织,伴纤维组织大量增生,同时组织细胞内可见吞噬的淋巴细胞。免疫组化显示组织细胞S-100强(+),CD68和lyzosyme(+);其余染色及标记均为阴性。结论喉结外Rosai-Dorfman病的病理诊断需与朗格汉斯细胞组织细胞增生症等鉴别,免疫组化染色寻找S-100蛋白阳性的组织细胞及其胞质内淋巴细胞的伸入运动是有效的鉴别方法。

Objective To study the clinicopathological features of Rosai-Dorfman disease.Methods A patient diagnosed as largngeal malignant tumor before operation by image examination was enrolled in this study.The clinical and image data were reviewed,and the lesion was examined by microscopy,special stains（PAS,Giemsa,Warthin-starry silver stain） and the immunohischemical staining for S-100,CD68,lyzosyme,SMA,P63,CD30,CD1a and Des.The related literatures were also reviewed.Results The patient was an elderly female,presented with a mass in the left half part of hypopharynx by CT.There was apophysis on some areas.The lesions involved in left superior section of glottis,epiglottis,left vocal cords and ring cartilage below about 6.5 mm.Histology showed that a large number of plasmacytes,lymphocytes and histiocytes were diffusely infiltrated in the lesions and surrounding strated muscles,with proliferation of fibrous tissue and lymphophagocytosis.Strongly positive immunoperoxidase staining for S-100,CD68 and lyzosyme was noted in the cytoplasm of most histiocytes,but the rest of the markers were all negative.Conclusions Histopathology and immunhistochemical staining confirm the diagnosis of Rosai-Dorfman disease in this case.The differential diagnosis includes Langerhan’s histiocytosis,chronic inflammation,reactive sinus hyperplasia,benign and malignant fibrous histiocytoma and plasmacytoma.Strongly positive immunoperoxidase staining for S-100 protein in the cytoplasm of tumor cells as well as lymphophagocytosis or emperipolesis are suggestive for the diagnosis.