Rosai-Dorfman disease: presentation, diagnosis, and treatment.

Abstract

Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease (RDD) is a rare benign disease with nodal and extranodal involvement. Herein, we report 3 cases in which the patients presented with nasal masses. In addition, 2 of the 3 patients had subglottic lesions. Only 2 of the 3 patients had cervical lymphadenopathy, which is the commonest presentation of RDD. Histopathology via fine-needle aspiration cytology (FNAC) or open biopsy is required for diagnosis. Emperipolesis with S-100 immunohistochemical staining is characteristic. The disease runs a benign course and the literature reports that treatment can vary from steroids, to cytotoxic chemotherapy to radiotherapy with variable outcomes. Surgical intervention may be necessary for airway protection. RDD is self-limiting, but can sometimes be a life-threatening condition. Treatment should be tailored to the individual patient.