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Case Reports
. 2025 Mar 25;61(4):585.
doi: 10.3390/medicina61040585.

Efficacy of Radiotherapy in Patients with Relapsing Primary Rosai-Dorfman Disease of the Nasal Cavity

Caius-Codrut Sarafoleanu  1   2 , Florentina-Carmen Badea  3 , Alina-Maria Georgescu  4   5 , Gabriela-Cornelia Musat  1   2 , Anica Andrei  6 , Ionut Tanase  1   2
Affiliations
Case Reports

Efficacy of Radiotherapy in Patients with Relapsing Primary Rosai-Dorfman Disease of the Nasal Cavity

Caius-Codrut Sarafoleanu et al. Medicina (Kaunas). .

Abstract

Rosai-Dorfman disease (RDD) is a rare proliferative disorder characterized by an overproduction of a type of immune cell called histiocytes, with unknown etiology. Although extranodal involvement is not uncommon, it is rarely seen within the nose. The available data are limited, and currently, there are no established guidelines for managing RDD. Different therapeutic options have been described, including corticosteroids, surgery, radiotherapy, or chemotherapy. This study aims to evaluate the potential benefit of radiotherapy in patients with nasal Rosai-Dorfman disease to improve the current diagnostic and therapeutic management. Herein, we report the case of a 54-year-old female patient with nasal RDD refractory to systemic corticosteroid therapy and transnasal endoscopic resection. She received fractionated low-dose radiotherapy with a total dose of 30 cGy administered in 2 cGy daily fractions. Complete remission was achieved, highlighting the potential role of radiotherapy as an effective therapeutic option for relapsing or steroid-refractory cases. This is one of the few well-documented cases reported with nasal Rosai-Dorfman disease treated with radiotherapy. Ongoing research on novel therapies offers hope for improved outcomes in relapsing cases that fail to respond to conventional treatments.

Keywords: Rosai–Dorfman disease; S100 protein; histiocytosis; radiotherapy; sinonasal.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
(a,b) Nasal endoscopy examination showing multiple submucosal swellings with a friable surface originating from the nasal septum and inferior turbinates in both nasal cavities, being more extensive on the left side.
Figure 2
Figure 2
(a,b) Computed tomography scans of the head in the coronal and axial planes showing multiple, homogeneous, hypo-intense polypoid masses, which narrowed the nasal passages more extensively on the left side, obstructing the left choanal arch, without sinus involvement or bone erosion. (c,d) Coronal and axial T1-weighted images showing iso-intense to slightly hypo-intense lesions compared to gray matter.
Figure 3
Figure 3
(a) HE 10×: Nasal-type mucosa showing massive inflammatory infiltrate consisting of numerous large pale histiocytes, small lymphocytes, and plasma cells. (b) HE20×: large histiocytes engulf intact small lymphocytes and plasma cells, a phenomenon called emperipolesis, which is fairly specific to RDD. (c) CD163 20×: The large histiocytes are positive for CD163. (d) S100 20×: The large histiocytes strongly co-express the S100 protein, which is characteristic of RDD.
Figure 4
Figure 4
Three-dimensional conformal radiotherapy plans in the coronal (a), axial (b), and sagittal (c) views. Gross tumor volume boost delivered to the blue-shaded area in the isodose curves.
Figure 5
Figure 5
(a,b) A 36-month follow-up nasal endoscopy showed no evidence of residual tumor or local recurrence with normal healing of the nasal mucosa.
See this image and copyright information in PMC

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