Zhao Xue, Yu Dan, Jin Chunshun
E.N.T.Department, the second hospital of Jilin University. Changchun City, Jilin Province, China.
Open Life Sci. 2018 Aug 21;13:263-268. doi: 10.1515/biol-2018-0032. eCollection 2018 Jan.
To summarize the etiology, pathology, diagnosis, clinical features, and treatment of the rare extranodal Rosai-Dorfman disease (RDD).
Clinical data of three cases of extranodal RDD who were admitted to the E. N. T. Department of the second hospital of Jilin University were analyzed retrospectively, and the literature was reviewed.
Three cases of extranodal RDD (maxillary sinus, nasal pharyngeal focus, and external auditory meatus) had a low fever and weight loss rather than painless enlarged lymph nodes. Surgical intervention was managed to clarify the diagnosis. All pathological diagnoses were extranodal RDD. Predisone for oral use was given to all patients postoperatively. There was no recurrence in the following 3 months, except case 1 was lost 2 months later.
RDD is a rare idiopathic histiocytic proliferative disorder defined by its unique histopathological features: a proliferation of huge histiocytic cells with emperipolesis and S-100(+), CD1a(-). RDD is characterized clinically by bilateral cervical painless enlarged lymph nodes, while extranodal RDD is rarer and its manifestations varied. A defined therapeutic regimen has not been elucidated. RDD in about 20% of patients is self-limited. Surgical intervention is the main management of treatment, with glucocorticoids used in initial medical therapy. More clinical trials are necessary before drawing conclusions.
总结罕见的结外Rosai-Dorfman病（RDD）的病因、病理、诊断、临床特征及治疗方法。
回顾性分析吉林大学第二医院耳鼻喉科收治的3例结外RDD患者的临床资料，并复习相关文献。
3例结外RDD（上颌窦、鼻咽部病灶、外耳道）均有低热、体重减轻，而非无痛性淋巴结肿大。通过手术干预明确诊断。所有病理诊断均为结外RDD。术后所有患者均口服泼尼松。除病例1在2个月后失访外，其余患者在随后3个月内均无复发。
RDD是一种罕见的特发性组织细胞增生性疾病，其独特的组织病理学特征为：大量组织细胞增生伴吞噬现象及S-100（+）、CD1a（-）。RDD临床上以双侧颈部无痛性淋巴结肿大为特征，而结外RDD较为罕见，表现多样。尚未阐明明确的治疗方案。约20%的患者RDD为自限性。手术干预是主要治疗手段，初始药物治疗使用糖皮质激素。在得出结论前需要更多的临床试验。
