Rare Etiology of a Common Symptom: Tracheal Obstruction Due to Extranodal Rosai–Dorfman Disease

1Virginia Commonwealth University, Richmond, VA

Meeting: Hospital Medicine 2013, May 16-19, National Harbor, Md.

Abstract number: 242

Case Presentation:

A 76‐year‐old woman with hypertension, type 2 diabetes mellitus, and a 20‐pack‐year smoking history presented with complaint of 6 weeks of nonproductive cough. Left breast cancer had been treated 7 years prior with surgery, chemotherapy, and radiation, without evidence of recurrence. Her cough had not improved with courses of antibiotics and oral corticosteroids prescribed by her primary care physician. A lateral chest radiograph revealed a 2‐cm nodule overlying the tracheal air column. Chest computed tomography revealed an intraluminal mass arising from the anterior tracheal wall, as well as mediastinal and left hilar lymphadenopathy. Recurrent breast cancer or new primary malignancy was suspected. Bronchoscopy revealed a tracheal mass 3 cm above the carina, causing near‐complete airway obstruction. The mass was debulked endoscopically. Pathologic examination of the mass revealed morphologic and immunologic findings consistent with Rosai–Dorfman disease. The patient's cough initially improved but recurred within months. Repeat computed tomography revealed a recurrent tracheal mass. She was treated with oral corticosteroids and radiotherapy, without sustained regression of the mass. She has since been managed with endoscopic tracheal mass debulking on several occasions, prompted by recurrent cough and dyspnea associated with serial regrowth of her tracheal mass.

Discussion:

Rosa–‐Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a histiocytic lymphoproliferative disorder of unknown etiology. The most frequent clinical presentation of RDD is massive, painless bilateral cervical lymphadenopathy, with fever, night sweats, and weight loss, more often affecting males, children and young adults, and people of African descent. Mediastinal, inguinal, and retroperitoneal nodes may also be involved. Extranodal RDD occurs in 30%–40% of cases. Although involvement of many different organs has been described, upper airway disease is rare, with only 2 case reports in the literature of tracheal obstruction due to extranodal RDD. The clinical course of RDD is unpredictable, with episodes of exacerbation and remissions that may last many years. In the majority of cases, RDD has a benign course, and treatment is not necessary. Therapy is required, however, for patients with extranodal RDD who have vital organ involvement. Treatment options include systemic corticosteroids, radiotherapy, and surgical debulking. Results with chemotherapeutic agents have not been encouraging.

Conclusions:

The purpose of reporting this case is to raise awareness of a rare presentation of an unusual, benign lymphoproliferative condition, the presenting symptoms and radiographic findings of which may raise false suspicion for malignancy.

To cite this abstract:

Wixted D. Rare Etiology of a Common Symptom: Tracheal Obstruction Due to Extranodal Rosai–Dorfman Disease. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 242. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/rare-etiology-of-a-common-symptom-tracheal-obstruction-due-to-extranodal-rosaidorfman-disease/. Accessed July 23, 2019.

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