Rosai-Dorfman disease presenting multiple intracranial lesions with uniquefindings on magnetic resonance imaging - Case report

Rosai-Dorfman disease (RDD) is a rare idiopathic histoproliferative disease affecting the systemic lymph nodes. Although an extranodal lesion has also been recognized, central nervous system involvement is extremely rare. To the authors' knowledge, only 20 cases of intracranial lesions have been rep orted previously. Intracranial RDD is clinically and radiologically difficu lt to distinguish from meningioma, and histological examination is essentia l for a definitive diagnosis. The authors treated a large frontal lobe tumo r associated with multiple meningeal nodules in a 67-year-old patient prese nting with diplopia and headache. Radiological examination indicated that t he mass was an inflammatory lesion rather than a meningioma. Microscopicall y the lesion consisted of mixed inflammatory infiltrate with marked emperip olesis, which is characteristic of RDD. A review of the literature and a di scussion of the differential diagnosis of this rare lesion are also present ed.