Rosai-Dorfman disease (RDD) was recognized as a distinct clinical entity by Rosai and Dorfman in 1969. It is a rare histiocytic proliferative disorder that occurs in various locations and occasionally involves the central nervous system. In this article, we aim to describe a case of intracranial RDD and to provide a review of the literature on intracranial RDD in Saudi Arabia. A 37-year-old woman presented with a history of generalized seizures. Physical examination disclosed bilateral cervical lymphadenopathy with no neurological deficit. Brain magnetic resonance imaging (MRI) demonstrated an extra-axial, homogenous, Gadolinium-enhancing, space-occupying lesion with extensive dural involvement. The patient was successfully treated by total surgical resection. Postoperatively, the patient did not receive any adjuvant therapy. Biopsy with immunohistochemical analysis confirmed the diagnosis of intracranial RDD. On follow-up examination, six months later, there was no recurrence of the lesion. A preoperative diagnosis of intracranial RDD is challenging since its MRI appearance can be similar to other intracranial diseases. Herein, we discussed some neuroradiographic findings that might help distinguish RDD from other intracranial diseases.
Keywords: case report; central nervous system; extranodal sinus histiocytosis with massive lymphadenopathy; meningioma; rosai-dorfman disease.
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