Rosai-Dorfman disease of the trachea: an extremely rare benign tumor

Rosai-Dorfman disease or sinus histiocytosis is a nonmalignant disease chiefly presenting as a painless cervical lymphadenopathy, although extranodal involvement is also frequent. Involvement of the tracheobronchial tree is extremely rare, and there are no clear guidelines on management. Rosai-Dorfman disease usually presents as a tracheal tumor, although it may also present with extraluminal compression. Only 12 cases have been reported in the English language literature. Histopathology is required to confirm the diagnosis. We describe what we think is India's first reported case of histologically proven tracheal Rosai-Dorfman disease in a 40-year-old otherwise healthy lady.