[Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of the airway: a case report and review of the literature]

Abstract

Objective: To improve the awareness of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) involving the airways.

Methods: The clinical presentations, endoscopic findings in the airways, pathological characteristics, and diagnosis and treatment of a case of Rosai-Dorfman disease was reported, and related literatures were reviewed.

Results: A 60-year-old female patient was admitted to this hospital because of recurrent wheezing for 18 months and aggravated for 1 month on March 6, 2007. The diagnosis of "bronchial asthma" had been made and oral prednisolone and inhaled budesonide resulted in symptom improvement. One month ago, she had wheezing again with inspiratory dyspnea, which was more obvious at recumbent position.She had been found to have high blood pressure and left adrenal adenoma 23 years ago, and as the diagnosis of "primary aldosteronism" was made but underwent no surgery. Left parotid gland tumor and left submandibular lymph nodes had been found, and surgical resection implemented in 1999. Lacrimal gland tumor resection of her eyes had been performed in 2000. Multiple subcutaneous nodules, rising and disappearing spontaneously, had been demonstrated in 2001. After admission, physical examination revealed nodules of 3.0 cm x 2.0 cm in her left submandibular area, and soybean sized nodules at both arms, back, chest, abdomen, buttocks and thighs. Chest CT scan and tracheal reconstruction showed that there were multiple nodules in the tracheal wall with narrow lumen, with no obvious enlargement of mediastinal lymph nodes. Lymph node biopsy showed faintly stained areas and the formation of plasma cells and lymphocytes of the deeply stained area, presenting as a sinus-like structure, and plasma cells and lymphocytes were engulfed in the plasma of the histiocytes, consistent with the diagnosis of Rosai-Dorfman disease.

Conclusions: Rosai-Dorfman disease involving the airway was a rare disease often misdiagnosed. Bronchoscopy was very helpful for the diagnosis. Histiocytosis with phagocytosis of plasma cells and lymphocytes was the pathological feature, and immunohistochemical staining positive for S100 protein and CD(68) was suggestive of the diagnosis. Surgical resection combined with corticosteroids or radiotherapy was effective treatment of the airway diseases.