Rosai-Dorfman Disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a rare disease caused by a benign proliferation of lymphocytes. Most patients present with painless adenopathy, most commonly in the cervical lymph nodes, but other sites have been described as well. Extra-nodal involvement is rare but most commonly affects the head and neck. Laryngeal and tracheal involvement has rarely been reported in the literature. We report a case of near complete obstruction of the distal trachea treated which was effectively treated with electrocautery snare, Xomed® microdebrider, and argon plasma coagulation.
A 77 year-old female with a medical history significant for breast cancer 7 years prior presented to her primary care physician with complaint of a persistent cough for 6 weeks. She denied any shortness of breath, dyspnea on exertion, fevers, chills, night sweats, or weight loss. Computerized tomography (CT) of her chest revealed a 2 cm intraluminal tracheal mass arising from the anterior wall of the trachea with associated mediastinal and hilar adenopathy. This was thought to represent recurrence of her breast cancer. She underwent flexible bronchoscopy revealing a spherical shaped mass with near complete occlusion of her tracheal lumen 3 centimeters above the carina. An electrocautery snare could only remove 50% of the mass. She was admitted to the hospital for definitive therapy. She underwent rigid bronchoscopy and a Xomed® microdebrider was used to debulk the tumor followed by argon plasma coagulation for hemostasis. The tumor was shaved down to the residual tracheal wall and it was noted intra-operatively that the tumor was invading the anterior and lateral walls of the trachea. Biopsy specimens taken at the time of the procedure were consistent with extra-nodal RDD. A repeat CT scan two months post-procedure revealed recurrence of the mass in the trachea with 25% intraluminal obstruction present despite a 3 month course of prednisone. She completed one course of 20 Gy external beam radiotherapy and follow up imaging reveals that current therapy has stabilized the growth of the tumor.
RDD is a rare lymphoproliferative disorder which usually causes painless adenopathy, but may present with symptoms caused by extra-nodal involvement. Most cases follow a relatively benign course with waxing and waning symptoms and do not require therapy. Treatment options described in the literature include corticosteroids, chemotherapy, low-dose radiation therapy, and surgical debridement. These treatments are usually only necessary with vital organ involvement or airway compromise. She was effectively treated with multi-modality therapy, including bronchoscopy and external beam radiation.
Central airway obstruction due to RDD is exceedingly rare, with only seven previous cases reported in the literature. Five of those cases were subglottic or laryngeal with extrinsic compression. We describe a case with an exophytic distal tracheal mass, which to our knowledge has not been described in the literature previously. This case is also unique in that the mean age of diagnosis is twenty years, with our patient presenting in her seventies. We successfully treated the obstruction with microdebridement, argon plasma coagulation, electrocautery and external beam radiation.
Keith Goulet, No Financial Disclosure Information; No Product/Research Disclosure Information