Rosai-Dorfman Disease: Rare Presentation as Isolated Mediastinal and Hilar Lymphadenopathy

Rosai-Dorfman disease (RDD) is a rare, benign, and predominantly nodal disease that most commonly presents as bilateral, painless cervical lymphadenopathy; although inguinal, axillary, mediastinal, and hilar lymphadenopathy has also been reported. Apart from nodal involvement, RDD has extranodal manifestations involving bone, soft tissue, and liver as well as constitutional symptoms of fever, night sweats, and weight loss, which make it reasonable to rule out the infectious, autoimmune, and malignant conditions as the differential diagnosis. We herein report a case of RDD affecting only the mediastinal and hilar region in an 83-year-old woman.