Three Cases of Extranodal Rosai-Dorfman Disease and Literature Review

OBJECT: To summarize the etiology, pathology, diagnosis, clinical features, and treatment of the rare extranodal Rosai-Dorfman disease (RDD). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.