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

Abstract

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.

Rosai-Dorfman disease with pulmonary involvement mimicking bronchogenic carcinoma

“…It can cause an exophytic mass either intraluminal or intramural. Patients usually present with symptoms related to bronchial compression [7]. Our case presented with the unique finding in radiology examination mimicking primary carcinoma of the lung due to massive mucin impaction within the main bronchus and its dilated branches.…”

“…Some studies suggest an association between RDD with pulmonary affection and IgG4 related diseases [2]. However, no Rosai-Dorfman disease involving the tracheobronchial tree is rarely reported in the literature [7]. The disease can involve larynx, subglottic area, trachea, and bronchi.…”

“…Some authors suggested the use of low-dose corticosteroids, methotrexate, or 6mercaptopurine in cases of extra-nodal involvement. The recurrence rate in RDD involving tracheobronchial airway had been reported, therefore long term follow up is recommended [7].…”