Rosai-Dorfman disease presenting in the oropharynx

We report a case of Rosai-Dorfman disease (RDD) presenting as an oropharyngeal mass, and we provide a detailed discussion of this rare clinical entity.

An initial biopsy, described as being consistent with lymphoma, was reviewed at a second outside institution and was thought to be reactive lymphoid hyperplasia. The patient then presented to our institution for a second opinion. Direct laryngoscopy revealed a firm 1- to 2-cm lesion involving the left soft palate and superior tonsillar pillar.

The diagnosis of RDD was confirmed with immunohistochemical staining on both cytology and histology. The patient elected conservative management and has remained asymptomatic for over 15 months.

The diagnosis of RDD may be challenging in cases presenting initially with extranodal disease. Close follow-up and repeat biopsies may be necessary. Identification of Rosai-Dorfman cells with emperipolesis and confirmation with appropriate immunohistochemical staining on both cytology and histology is diagnostic of RDD. Conservative treatment is appropriate in select cases.