摘要目的 探讨气管原发窦组织细胞增多症(Rosai-Dorfman病,RDD)的临床表现、病理特点、免疫组织化学染色结果及鉴别诊断,提高对气管原发性RDD的认识.方法 分析1例术前诊断为气管恶性肿瘤病例的临床病史、支气管镜检查及影像学资料、组织病理学形态、免疫组织化学标志物检测,并结合相关文献进行复习.以“Rosai-Dorfman病”或“窦组织细胞增生症”为关键词通过万方数据库进行检索；以“Rosai-Dorfman disease”、“sinus histiocytosis”、“trache”和“lung”为关键词通过PubMed数据库进行检索,检索时间截至2012年7月.结果 患者男,63岁,因“气管内肿物”收入院.胸部CT示气管右侧占位性病变,增强扫描强化明显,密度欠均匀；气管镜下见气管下段新生物突入管腔,新生物表面黏膜完整,气管腔呈缝隙状,支气管镜无法通过,行局部气管切除术.术后镜下组织学表现为明区和暗区,暗区为气管黏膜组织中可见纤维组织增生,大量浆细胞、淋巴细胞及组织细胞弥漫浸润,累及气管壁深层,气管软骨受侵蚀.明区为较大组织细胞,呈椭圆形或多角形,胞质粉染,可见吞噬的淋巴细胞和浆细胞.免疫组织化学染色示大组织细胞胞质S-100蛋白及CD68均阳性,确诊为气管原发性RDD.患者术后未给予特殊治疗,随访至今未复发.在万方医学数据库和PubMed数据库上检索到呼吸系统原发性RDD的国内外文献13篇,病例26例,其中以发生于上呼吸道多见(21例)；肺原发性RDD 4例；累及气管的RDD国内外文献5篇,病例5例；气管原发性RDD仅1例报道,患者女,39岁,呼吸困难1个月,误诊为支气管哮喘,给予糖皮质激素等治疗无效,胸部CT气管隆突上方见不规则肿块,气管阻塞86％.为防止患者发生窒息,行气管环袖状切除和再吻合术,术后依据病理组织学和免疫组织化学染色确诊为气管RDD.术后未接受任何治疗,随诊12个月未复发.结论 气管原发性RDD较罕见,呼吸困难是其临床重要表现,手术完全切除,预后较好.确诊主要依靠典型的组织病理学、免疫组织化学染色及鉴别诊断,免疫组织化学染色S-100蛋白、CD68阳性的大组织细胞及胞质内细胞的伸入现象是诊断RDD的重要依据.应与其他可发生于气管的良恶性病变进行鉴别诊断.

abstractsObjective To study the clinicopathological features,diagnosis and prognosis of primary sinus histiocytosis (Rosai-Dorfman disease,RDD)of the trachea by case report and review of the literature.Methods A 63 year old man with a space-occupying lesion of the trachea firstly diagnosed as a malignant tumor was admitted to this hospital for further evaluation and treatment.The lesion was removed by surgery and the final diagnosis was primary RDD.The clinical data of the case was analyzed and the related literatures were reviewed.The literature review was carried out respectively with" Rosai-Dorfman disease"and "sinus histiocytosis"as the key words in Wanfang Med Online and with" Rosai-Dorfman disease"," sinus histiocytosis"," trachea or lung" as the key words in PubMed database by July 2012.Results The chest computerized tomography of the case showed that the mass was located at the right side of the trachea with heterogeneous density and contrast enhancement.Bronchoscopy revealed a neoplasma occluding the distal trachea.The lesion was excised by surgery.Microscopic histology showed that in the dark-staining area a large number of lymphocytes and plasma cells were noted while the light-staining area was formed by giant histiocytes.The pathological changes invaded the tracheal wall and eroded the cartilages.Intact lymphocytes and plasma cells were observed within the eosinophilic cytoplasm of the histiocytes.Immunohistochemistry showed that the giant histiocytes were strongly positive for S-100 protein and CD68 protein.Primary RDD of trachea was confirmed.The patient remained well without any other treatment or evidence of progression for 11 months.A total of 13 literatures and 26 cases were retrieved from Wanfang Med Online and Pubmed,including 21 cases of primary RDD of the upper respiratory tract and 4 cases of primary RDD of the lung.A total of 5 literatures and 5 cases of RDD affecting the trachea were retrieved from Wanfang Med Online and Pubmed.There was only one case of primary RDD of the trachea in Pubmed.A 39-year-old female patient with 1 month of dyspnea was misdiagnosed as having bronchial asthma and was unresponsive to empirical corticosteroid and bronchodilator therapy.The chest computerized tomography revealed an ill-defined irregular soft tissue in the trachea.A tracheal ring sleeve resection and reanastomosis was performed to prevent asphyxia.The mass was confirmed to be primary RDD of the trachea according to histopathology and immunohistochemistry.The patient was well without any treatment for 12 months.Conclusions Primary RDD of the trachea is an extremely rare disease,with dyspnoea as a feature of the disease.When it is completely removed,the prognosis is good.Typical histopathology and immunohistochemistry are needed to make a definite diagnosis.The positive immunohistochemistry staining for S-100 and CD68 protein in giant histiocytes and lymphocyteemperipolesis are essential for the diagnosis.The differential diagnosis includes other benign or malignant space-occupying lesions of the trachea.