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Tracheal Rosai-Dorfman Disease

Imaging findings

Chest radiograph and CT demonstrate a focal, nodular, soft-tissue mass extending into the tracheal lumen in the right paratracheal region, associated with mild right paratracheal thickening.

Key takeaways

Biopsy of the endotracheal mass confirmed Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy). While Rosai-Dorfman disease is primarily a benign lymphoproliferative disorder presenting with massive painless cervical lymphadenopathy, extranodal involvement occurs in a minority of cases, and isolated tracheal or upper airway involvement is exceedingly rare, often mimicking malignant airway neoplasms such as squamous cell carcinoma, adenoid cystic carcinoma, or lymphoma.

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