Intracranial meningeal Rosai-Dorfman disease mimicking multiple meningiomas: Three case reports and a literature review.

BACKGROUND: Rosai-Dorfman disease (RDD) is a benign, self-limiting and non-neoplastic histiocytic proliferative disorder with a poorly defined pathogenesis. Central nervous system involvement is extremely rare, particularly in cases with multiple intracranial masses. There is no consensus on the optimal treatment and management of RDD. CASE DESCRIPTION: The first case was of a 39-year-old man who presented with headaches and paroxysmal convulsions of the limbs. Magnetic resonance imaging (MRI) 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. The second case was of a 53-year-old man admitted with epileptic seizures. An MRI revealed multiple homogeneously enhancing masses in the left parietal, temporal, and occipital meningeal regions. The largest mass was surgically removed and histopathologic examination confirmed and RDD diagnosis. The third case was of a 9-year-old girl admitted with bilateral exophthalmos and incomplete eyelid closure. MRI displayed multiple heterogeneously enhancing masses in the right parietal meningeal region. All masses were surgically removed, and the histopathologic diagnosis was RDD. CONCLUSIONS: RDD with multiple isolated intracranial foci is rare but should be considered as a differential diagnosis when multiple meningiomas are suspected in children and adults. This short series suggests that preoperative diagnosis is challenging, and the definitive diagnosis requires immunohistochemical examination. Currently, surgical resection is the most effective therapy for the rapid relief of symptoms.