Intracranial Meningeal Rosai-Dorfman Disease Mimicking Multiple Meningiomas: 3 Case Reports and a Literature Review.

Abstract

Rosai-Dorfman disease (RDD) is a benign, self-limiting, and nonneoplastic histiocytic proliferative disorder with a poorly defined pathogenesis. Central nervous system involvement is extremely rare, particularly cases with multiple intracranial masses. There is no consensus on optimal treatment and management of RDD. In case 1, a 39-year-old man presented with headaches and paroxysmal convulsions of the limbs. Magnetic resonance imaging showed multiple solid masses located at the right frontoparietal and left frontal meningeal regions. The masses were surgically removed for staging, and the histologic diagnosis was consistent with RDD. In case 2, a 53-year-old man was admitted with epileptic seizures. Magnetic resonance imaging revealed multiple, homogeneously enhancing masses in the left parietal, temporal, and occipital meningeal regions. The largest mass was surgically removed, and histopathologic examination confirmed RDD. In case 3, a 9-year-old girl was admitted with bilateral exophthalmos and incomplete eyelid closure. Magnetic resonance imaging displayed multiple, heterogeneously enhancing masses in the right parietal meningeal region. All masses were surgically removed, and the histopathologic diagnosis was RDD. RDD with multiple isolated intracranial foci is rare but should be considered in the differential diagnosis when multiple meningiomas are suspected in children and adults. Preoperative diagnosis is challenging, and definitive diagnosis requires immunohistochemical examination. Surgical resection is the most effective therapy for rapid relief of symptoms.