Rosai-Dorfman disease isolated to the central nervous system: a report of six cases.

We reviewed the diagnosis and treatment of six patients with CNS Rosai-Dorfman disease (RDD). Lesions were located in the cerebral convexity, middle cranial base, parasaggital, petrous orbit, and thoracic spine. Preoperatively, all the lesions were misdiagnosed as meningioma. Histopathology of all CNS lesions showed a characteristic feature called emperipolesis, where small lymphocytes or plasma cells were engulfed in histiocyte cytoplasm. Total resection of lesions was performed in all cases, and at an average follow-up of 15 months, all patients are alive and well with no evidence of recurrence. Preoperative diagnosis of CNS RDD is challenging. Surgical removal of lesions is an effective treatment. More research is needed to clarify the effectiveness of other treatment options such as radiosurgery and corticosteroid therapy.