Rosai-Dorfman disease of the lung with features of obliterative arteritis

Abstract

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare benign non-Langerhans cell histiocytosis. Twenty-five to forty percent of the cases are extranodal and have been reported in virtually all anatomic locations. In this article, we report a highly unusual case of RDD which presented as multiple pulmonary nodules and associated hilar lymphadenopathy. On resection, extensive obliterative arteritis was noticed. This pathologic presentation of RDD has not been reported before in the English literature. Accurate recognition of this entity is crucial to prevent unnecessary aggressive treatment.

Unusual Presentation of Rosai-Dorfman Disease: Report of a Rare Case

“…A histopathology report was positive for histiocytes with emperipolesis, and further immunohistochemical staining was positive for S-100 protein, which changed the diagnosis from suspected malignancy to RDD. At 6-month follow-up after surgery, the patient was free of additional symptoms and there was no evidence of new chest lesions on imaging [10]. Case reports have many shown different results regarding treatment – some cases respond to corticosteroids or other immunosuppressive medication, and others show response to immunomodulatory agents, as in our case.…”