鼻腔鼻咽部的淋巴结外Rosai-Dorfman病的诊治分析

Diagnosis and Treatment of Extranodal Rosai-Dorfman Disease in Nasal Cavity and Nasopharynx

目的探讨鼻腔及鼻咽部淋巴结外Rosai-Dorfman病的临床表现、诊断、治疗及预后。方法对2009年11月-2013年8月就诊的4例鼻腔及鼻咽部的淋巴结外Rosai-Dorfman病的患者资料进行总结、分析。结果 4例患者均行外科手术治疗,完整切除病变。病理结果示：增生的组织细胞体积大,并见淋巴细胞＂包入＂。免疫组织化学细胞呈CD68（＋）、S-100（＋）、CD1a（-）。跟踪随访28-45个月,平均35个月,4例均无复发。结论 Rosai-Dorfman病是罕见、病因不明的疾病,目前尚无公认的治疗方案。手术治疗是较为成功的治疗选择;在保证完全切除病变的前提下,微创手术是首选。

Objective To investigate the clinical manifestations,diagnosis,treatment and prognosis of RosaiDorfman disease（RDD） in nasal cavity and nasopharynx.Methods We retrospectively analyzed the clinical data of4 patients with extranodal RDD admitted to the Department of Otolaryngology,Head and Neck Surgery of our hospital between November 2009 and August 2013.Results All patients received complete surgical removal of the lesions.Histopathological examination showed that large histocytes had an abundant foamy cytoplasm containing ingested lymphocytes as a result of emperipolesis,and were strongly positive for S-100 and CD68 on immunohistochemical analysis,but negative for CD1 a.All patients were followed up for 28 to 45 months（averaging 35 months）,and no recurrence was observed during the follow-up period.Conclusions RDD is a rare disease,and the exact etiology and pathogenesis of RDD are not fully understood.There is no unified treatment plan recommended,and complete surgical removal of the lesions is an effective method.Minimally invasive surgery may be a good choice for treatment of this disease.