Abstract

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare benign clinical disorder that occurs in all age groups and is prevalent in children and adolescents. The most frequent clinical presentation consists of significant bilateral cervical lymphadenopathy that resolves spontaneously. Naso-sinus involvement is described in 11% while neurological involvement is present in less than 5% of cases; neurological presentation in the form of an isolated brain tumor is rare. Here we report two cases of this rare disease the first one represents a naso-sinus and dental involvement. Therapeutically the patient was started on Prednisone 1 mg /kg. The 2nd represents a much rarer involvement which is the isolated central neurological. A total excision was done.
Treatment of Rosai-Dorfman disease, when indicated, is not codified. Depending on the case, it includes surgery, corticosteroids, immunosuppressants and/or alpha interferon.