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Sinonasal presentations of Rosai–Dorfman disease: a scoping review

Published online by Cambridge University Press:  30 July 2025

Kwasi Ansere Ofori
Affiliation:
School of Public Health, Yale University, New Haven, CT, USA
Ryan A. Rimmer*
Affiliation:
School of Medicine - Division of Otolaryngology, Yale University, New Haven, CT, USA
*
Corresponding author: Ryan A. Rimmer; Email: ryan.rimmer@yale.edu
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Abstract

Objectives

Rosai–Dorfman disease is a rare histiocytic disorder typically presenting with cervical lymphadenopathy. Sinonasal involvement is uncommon and presents diagnostic and therapeutic challenges. This scoping review synthesises literature on the clinical presentation, diagnosis, management and outcomes of sinonasal Rosai–Dorfman disease.

Method

We systematically searched PubMed, Scopus and Embase. Articles were screened using Endnote. Studies reporting sinonasal Rosai–Dorfman disease were included. The review followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses-ScR (Scoping Review) guidelines.

Results

Thirty studies comprising 36 patients were included. Common symptoms were nasal obstruction (80.6 per cent) and epistaxis (41.7 per cent). Computed tomography (75 per cent) and magnetic resonance imaging (36.1 per cent) were primary imaging modalities. Histopathology showed emperipolesis (66.7 per cent), S-100 (69.4 per cent) and CD68 (47.2 per cent) positivity. Management was mainly surgical (72.2 per cent), with corticosteroids (44.4 per cent), radiotherapy (5.6 per cent) and chemotherapy (5.6 per cent) used less frequently. Outcomes included complete resolution (38.9 per cent), stable disease (38.9 per cent) and recurrence (16.7 per cent).

Conclusion

Diagnosis relies on histopathology and imaging. Surgical procedures, often with corticosteroids, remain the primary treatment. Future research should guide diagnostic and treatment protocols.

Information

Type
Review Article
Creative Commons
Creative Common License - CCCreative Common License - BY
This is an Open Access article, distributed under the terms of the Creative Commons Attribution licence (http://creativecommons.org/licenses/by/4.0), which permits unrestricted re-use, distribution and reproduction, provided the original article is properly cited.
Copyright
© The Author(s), 2025. Published by Cambridge University Press on behalf of J.L.O. (1984) LIMITED.
Figure 0

Table 1. Patient characteristics

Figure 1

Table 2. Patient clinical presentations/symptoms

Figure 2

Figure 1. Venn diagram illustrating the use and overlap of imaging modalities in the diagnosis of sinonasal Rosai–Dorfman disease. CT = computed tomography; MRI = magnetic resonance imaging; PET-CT = positron emission tomography.

Figure 3

Figure 2. Venn diagram showing the overlap of treatment strategies used across sinonasal Rosai–Dorfman disease cases, including surgery, corticosteroids, chemotherapy and radiotherapy.