Rosai-Dorfman disease presenting with multiple orbital and intracranial masses

Abstract

Rosai-Dorfman disease is an idiopathic histocytic proliferative disorder typically characterized by painless cervical adenopathy, fever, and weight loss. Extranodal manifestations are responsible for presentation in approximately 25% of patients. Orbital involvement has been described in about 10% of patients. There have been only 16 reported cases of Rosai-Dorfman disease presenting with an intracranial mass. We report an unusual case of a patient presenting with bilateral orbital tumors as well as multiple intracranial masses. Clinical, magnetic resonance imaging, and histopathological features are discussed.