Exophytic Tracheal Mass. A Rare Presentation of Rosai–Dorfman Disease

A 77-year-old female with a history of previously treated breast cancer presented with intractable cough for 6 weeks and no other associated symptoms. Physical examination disclosed coarse breath sounds throughout both lung fields, but no wheezing or stridor. Routine laboratory studies were notable only for an elevated white blood count of 12,400/ml with 86.3% neutrophils. Computed tomographic chest imaging (Figure 1) showed a roughly spherical exophytic mass arising from the anterior wall of the trachea with near occlusion of the airway. Associated mediastinal and left hilar adenopathy was also observed. Given the history of breast cancer, metastatic disease was considered a strong possibility. A primary tracheal malignancy was also considered as part of the differential diagnosis. As the patient was stable, flexible fiberoptic bronchoscopy was performed in the bronchoscopy suite under moderate sedation. The lesion was visualized in the mid-trachea (Figure 2). An electrocautery snare (Olympus, Center Valley, PA) was then used to ablate and debulk the mass (Figure 3). The patient tolerated the procedure uneventfully. The next day, she was taken to the operating room. With the assistance of an anesthesiologist, she underwent direct intubation with a rigid tracheoscope (BryanDumon 12/11 mm; Bryan Corporation, Woburn, MA) under general anesthesia. Jet ventilation was used throughout the procedure. The remaining tumor was cored out with the rigid scope. A Xomed microdebrider (Medtronic, Minneapolis, MN) was used to further shave down the tumor along the anterior and lateral walls of the trachea. Argon plasma coagulation was used on the residual tumor surface to achieve coagulation (Figure 4). There was no bleeding and the patient was extubated uneventfully. Histological examination of biopsy samples showed that the mass consisted predominantly of large histiocytes with ample finely vacuolated eosinophilic cytoplasm. A mixed inflammatory infiltrate consisting of lymphocytes, plasma cells, and neutrophils was interspersed in between the large cells. Emperipolesis was noted (Figure 5). Immunohistochemical stains were positive for CD68 and S-100 (Figure 5) and negative for pan-cytokeratin AE1/AE3 and pan-melanoma cocktail. The morphology and immune profile of the mass confirmed a diagnosis of Rosai–Dorfman disease. Adjunct treatment with prednisone (60 mg daily for 2 wk) was initiated and she was discharged home. Repeat imaging 5 months later showed that the tumor had regrown. Low-dose radiation therapy (20 Gy