Extranodal Rosai-Dorfman Disease Presenting as an Intranasal Mass

Abstract

Objective. We report the case of a 40-year-old female with biopsy-proven cutaneous extranodal Rosai-Dorfman who presented with symptoms of nasal obstruction and sinusitis. Rosai-Dorfman disease, also known as sinus histiocystosis with massive lymphadenopathy (SHML), is a rare, idiopathic disorder characterized by a benign proliferation of histiocytes initially thought to be limited to cervical lymph nodes. However, between 30% and 50% of patients identified with SHML have extranodal manifestations. Study design. Case report and literature review. Results. The patient presented to the otolaryngology clinic with nasal obstruction. A CT scan demonstrated lesions of the left inferior turbinate and anterior nasal septum with bony erosion. The patient underwent a biopsy of the nasal mass. The specimen was evaluated with flow cytometry, microscopy, and stained for immunohistochemical markers which confirmed a diagnosis of Rosai-Dorfman. Conclusion. Rarely, Rosai-Dorfman disease may manifest in the nasal cavity with symptoms mimicking that of sinusitis. Otolaryngologists should be familiar with the pathophysiology of the disease, the variability of extranodal involvement, radiologic findings, and the immunohistochemical analysis of Rosai-Dorfman to aid in diagnosis. This report emphasizes the radiological, pathological, and clinical appearance of the disease.