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Rosai-Dorfman disease: presentation, diagnosis, and treatment
Kwong Lun Lai1, Victor Abdullah, Kwan Shun Ng
1Department of Ear, Nose and Throat, United Christian Hospital, Hong Kong Special Administrative Region, China.
Background:
Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease (RDD) is a rare benign disease with nodal and extranodal involvement.
Methods And Results:
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.
Conclusion:
RDD is self-limiting, but can sometimes be a life-threatening condition. Treatment should be tailored to the individual patient.
