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, 18 (3), e158-62

Radiotherapy for Steroid-Resistant Laryngeal Rosai-Dorfman Disease

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Radiotherapy for Steroid-Resistant Laryngeal Rosai-Dorfman Disease

D Toguri et al. Curr Oncol.

Abstract

Rosai-Dorfman disease is a rare lymphoproliferative disorder that can have nodal and extranodal manifestations. In the absence of established guidelines for the management of this condition, various therapeutic modalities are used, including radiotherapy. Radiation dosages and fractionation schedules have not been reported in all instances. We present a case in which glottic and subglottic Rosai-Dorfman lesions causing airway obstruction in a frail steroid-refractory patient were put into complete remission using radiotherapy. The lesions responded transiently to a course of prednisone, but responded completely to external-beam radiation, with minimal side effects to the patient.

Keywords: Rosai–Dorfman disease; histiocytosis with massive lymphadenopathy; radiotherapy.

Figures

FIGURE 1
FIGURE 1
Images obtained by flexible laryngoscopy: (A) right-sided vocal cord lesion causing asymmetrical vocal cord movement; (B) exophytic nasopharyngeal mass.
FIGURE 2
FIGURE 2
Histology micrographs demonstrating classical features of Rosai–Dorfman disease: (A) histiocytosis, expanded paracortex, and reduced number of follicles with germinal centers; (B) emperipolesis; (C) positive staining for the CD68, CD163, and S-100 immunophenotypes.
FIGURE 3
FIGURE 3
Three-dimensional conformal radiotherapy plans in the (A) axial and (B) sagittal views. Gross tumour volume boost delivered to the blue-shaded area in the isodose curves.
FIGURE 4
FIGURE 4
Computed tomography scans of the head and neck in the axial plane showing right-sided Rosai–Dorfman lesion of the subglottis (A,B) before and (C,D) after radiation therapy.

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