CNS Manifestations of Rosai-Dorfman Disease

Rosai-Dorfman disease (RDD) is an abnormal proliferation of histiocytes which manifest classically as bilateral cervical lymphadenopathy and B symptoms. Rarely, it also presents with involvement of other systems. CNS RDD is extremely rare and accounts for 5% of reported cases. The clinical picture is dependent on the area of CNS affected. It is mostly diagnosed on MRI, however, it may be confused with a meningioma, dural based metastases, lymphoma, sarcoidosis, etc. Diagnosis is based on typical histopathological features. The recommended treatment for symptomatic CNS manifestations of RDD is complete surgical resection.