摘要目的 探讨上呼吸道结外Rosai-Dorfman病的临床病理特征及鉴别诊断.方法 采用光镜及免疫组织化学LSAB法结合临床资料,对10例发生于上呼吸道的结外Rosai-Dorfman病进行临床病理学分析.结果 10例患者中,男性3例,女性7例,发病年龄20 ～61岁,平均38岁.肿瘤分别位于鼻腔鼻窦(7例)、鼻咽(2例)和硬腭气管(1例).患者多以鼻塞、鼻衄或鼻腔鼻咽新生物就诊.CT显示鼻腔鼻窦等相应部位占位性病变,无骨质破坏.组织学显示肿瘤由相互交错的淡染区与暗染区组成,淡染区见大圆形或多边形的组织细胞,部分组织细胞见吞噬现象；暗染区由聚集的淋巴细胞和浆细胞组成.免疫组织化学染色显示组织细胞弥漫强阳性表达S-100蛋白,部分表达CD68等.随访6例,经手术治疗均健在,无复发.结论 上呼吸道结外Rosai-Dorfman病较为少见,多发生于鼻腔鼻窦,形态学上易与鼻硬结病相混淆,常需免疫组织化学染色加以鉴别.

abstractsObjective To study the clinicopathologic features and differential diagnosis of extranodal Rosai-Dorfman disease (RDD) of the upper respiratory tract.Methods The clinical,pathologic and immunohistochemical features of 10 cases of RDD were evaluated.Results Among the 10 cases studied,there were 3 males and 7 females.The age of patients ranged from 20 to 61 years old ( mean 38 years).The lesion arose in the nasal cavity (7 cases),nasopharynx (2 cases) or hard palate to trachea ( 1 case).Most of the patients presented with nasal obstruction,rhinorrhagia or tumor mass in the nasal / nasopharyngeal regions.CT scan often showed the presence of soft tissue lesion without bone destruction.Histologically,extranodal RDD was characterized by light-staining bands alternating with dark-staining bands.The light-staining bands were formed by aggregates of large round or polygonal histiocytes with emperipoiesis. The dark-staining bands were formed by abundant lymphoplasmacytic infiltrates.Immunohistochemical study showed that the histiocytes strongly expressed S-100 protein and partially expressed CD68.Six patients had no recurrence after surgical resection.Conclusions Extranodal RDD of the upper respiratory tract is a rare disorder of histiocytic proliferation,which usually involves the nasal cavity and paranasal sinuses. RDD can easily mimic rhinoscleroma, mainly due to the overlapping morphologic appearance.Immunohistochemical study is helpful in the differential diagnosis.