CASE CONFERENCES The Proceduralist Section Editor: George Eapen, M.D.

Exophytic Tracheal Mass A Rare Presentation of Rosai–Dorfman Disease Aamer Syed1, Rajiv Malhotra1, Samira Shojaee1, and Ray W. Shepherd1 1

Section of Interventional Pulmonology, Division of Pulmonary and Critical Care Medicine, Virginia Commonwealth University Medical Center, Richmond, Virginia

Computed tomographic imaging of the chest for evaluation of a persistent cough led to the unexpected discovery of a large endoluminal mass in the mid-trachea. Biopsies revealed Rosai–Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy. How should this rare condition be managed? 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

Figure 1. (A and B) Computed tomographic scan of chest showing an exophytic mass within the tracheal lumen (arrows).

(Received in original form April 24, 2013; accepted in final form May 11, 2013 ) Correspondence and requests for reprints should be addressed to Aamer Syed, Virginia Commonwealth University, Pulmonary Critical Care, 1200 East Broad Street, P.O. Box 980050, Richmond, VA 23298. E-mail: [email protected] Ann Am Thorac Soc Vol 10, No 5, pp 518–520, Oct 2013 Copyright © 2013 by the American Thoracic Society DOI: 10.1513/AnnalsATS.201304-096OT Internet address:


AnnalsATS Volume 10 Number 5 | October 2013

CASE CONFERENCES debulking via rigid bronchoscopy was performed in two settings because of the large tumor size. Complete debridement of the tumor was achieved with a combination of endotracheal Nd:YAP (neodymiumdoped yttrium aluminum perovskite) laser and mechanical debulking.


Figure 2. Intraluminal mass in trachea.

divided over 10 doses) was initiated with resultant reduction in the size of the tumor. The lesion demonstrated stability for the next 2 years but recurred to its original size,

Figure 3. Initial debulking of mass.

Case Conferences: The Proceduralist

resulting in hemoptysis, significant dyspnea, and respiratory failure. Emergency bronchoscopy revealed showed more than 90% occlusion of the tracheal lumen. Tumor

Sinus histiocytosis with massive lymphadenopathy was first reported as a disease entity by the pathologists Rosai and Dorfman in 1969 and has since been referred to as Rosai–Dorfman disease. The cause of this rare, nonmalignant histiocytic disorder is unknown, although it has been associated with certain viruses including Epstein–Barr virus and human herpesvirus 6. The disease may be more common in males and in individuals of African descent. Rosai– Dorfman disease often presents in teenagers or young adults as painless cervical lymphadenopathy, which may be accompanied by fever, night sweats, and weight loss. Other nodal sites of involvement include the mediastinum and retroperitoneal, axillary, and inguinal regions. Infiltrative lesions of the head and neck are commonly encountered and may involve the nasal cavity, salivary glands, eyes, and retro-orbital regions. Lytic bone lesions, pulmonary nodules, and skin or subcutaneous tissue lesions have also occasionally been described. Laboratory evaluation may show leukocytosis, polyclonal hypergammaglobulinemia, anemia, and elevated erythrocyte sedimentation rate. The diagnosis is made on biopsy of affected tissue. Histological examination of involved lymph nodes typically reveals an abnormal proliferation of large histiocytes including dendritic cells closely associated with macrophages, lymphocytes, and plasma cells. In the appropriate clinical and pathological setting, the presence of emperipolesis (the engulfment of erythrocytes and lymphocytes by histiocytes that express S-100) is considered diagnostic for the disease. Immunohistochemical stains are also characteristically positive for CD68. Unless vital structures or organs are compromised, patients usually have a good prognosis and spontaneous resolution is frequently observed. No curative treatment modality has been established; however, responses to glucocorticoid therapy and 519


Figure 4. Patent lumen after complete debulking.

external beam radiation have been reported. Chemotherapy has also been employed in selected instances.

Presentation limited to the mediastinum is rare. Only seven cases of tracheal involvement have been reported.

Of those, five were subglottic or laryngeal in location and associated with compromise of the airway. Repeat mechanical and thermal tumor debulking has been used on occasion to maintain patency of the airway. It is remarkable that our patient’s presenting symptoms were limited to a nonresolving cough despite the degree of tracheal obstruction. Given her overall stability, we were able to perform our initial bronchoscopy under moderate sedation without the need for endotracheal intubation although we were equipped to deal with an unanticipated airway emergency in the bronchoscopy suite. Initial electrocautery was useful for opening the airway and reducing the risk of uncontrolled bleeding in anticipation of subsequent tissue debulking via rigid bronchoscopy. Repeat radiation therapy was considered, but was limited by prior radiation exposure from her treatment for breast cancer.

Follow-Up After the most recent endotracheal Nd:YAP laser and mechanical debulking of the procedure, the patient recovered and returned home from the hospital. She continues to be monitored closely with repeat imaging every 6 months after the initial diagnosis was made nearly 5 years ago. n Author disclosures are available with the text of this article at

Figure 5. Large cells with ample, finely vacuolated, eosinophilic cytoplasm. Emperipolesis (arrow). S-100 stain; original magnification, 340.

Recommended Reading Favara BE, Feller AC, Pauli M, Jaffe ES, Weiss LM, Arico M, Bucsky P, Egeler RM, Elinder G, Gadner H, et al.; WHO Committee on Histiocytic/Reticulum Cell Proliferations; Reclassification Working Group of the Histiocyte Society. Contemporary classification of histiocytic disorders. Med Pediatr Oncol 1997;29:157–166. Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease): review of the entity. Semin Diagn Pathol 1990;7:19–73.


Komp DM. The treatment of sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease). Semin Diagn Pathol 1990;7:83–86. Levine PH, Jahan N, Murari P, Manak M, Jaffe ES. Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease). J Infect Dis 1992;166:291–295. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity. Arch Pathol 1969;87:63–70. Sennes L, Koishi H, Cahali R, Sperandio F, Butugan O. Rosai–Dorfman disease with extranodal manifestation in the head. Ear Nose Throat J 2004;83:844–847.

AnnalsATS Volume 10 Number 5 | October 2013

Exophytic tracheal mass. A rare presentation of Rosai-Dorfman disease.

Exophytic tracheal mass. A rare presentation of Rosai-Dorfman disease. - PDF Download Free
1MB Sizes 0 Downloads 0 Views