Nasal Presentations of Rosai-Dorfman Disease: Clinical Manifestation and Treatment Outcomes.

PURPOSE: There has been a lack of evidence-based management strategies on the nasal presentations of Rosai-Dorfman disease (RDD). We aim to investigate the clinical manifestation, treatment, and outcomes in patients with nasal RDD.

METHODS: We retrospectively reviewed available medical records of patients diagnosed with nasal RDD from 2014 to 2021 at our department.

RESULTS: A total of 26 patients were included with a marked female preponderance (2.25:1). The most common symptom and affected sites were nasal congestion (31%) and nasal cavity (73%), respectively. The average times of biopsies was 1.5 times (range: 1-3). The histiocytes were positive about S100 and CD68 and negative for CD1a with common emperipolesis. The mean duration of follow-up was 34 months (range, 3-87). One patient with concomitant nasal small B-cell lymphoma achieved complete remission after chemoradiotherapy. Recommended treatments were endoscopic resection (92%) and oral corticosteroids (21%). Surgery was performed to remove the resectable lesion as completely as possible. Corticosteroids induced almost 100% overall remission. Of the relapses, two patients achieved an overall response and one remained in progressive stage after subsequent excision. Two patients only received dissection biopsy that responded to oral corticosteroid administration and combined therapies of lenalidomide and dexamethasone, respectively.

CONCLUSIONS: Diffuse lesions in nasal cavity and sinuses, and even widely affected nasal skull base, laryngopharynx, orbit, and cavernous sinus, should be considered the possibility of Rosai-Dorfman disease. Characteristic immunohistochemical staining is helpful for the diagnosis. Endoscopic surgical therapy remains the mainstream treatment for patients enduring an unbearable course. Oral corticosteroid administration serves as an adjuvant therapy for first-line treatments.