Intracranial Rosai-Dorfman Disease - a Case Report and a Review of the Literature

Rosai-Dorfman disease (RDD) is an uncommon, non-neoplastic benign lymphoproliferative disease characterized by prominent, painless cervical lymphadenopathy with fever and leukocytosis. RDD is histologically characterized by emperipolesis, where large histiocytes become infiltrated with lymphocytes and plasma cells. Intracranial RDD is extremely rare. Only 79 cases have been reported, including the present case. Intracranial RDD is associated with headaches, seizures, and numbness caused by increased intracranial pressure. A 67-year-old Japanese woman presented with dizziness and was diagnosed with a tumor in the cerebral falx. The preoperative radiological diagnosis was meningioma. She had no lymphadenopathy. The patient underwent a craniotomy and tumor resection. The tumor consisted of lymphoid tissue with scattered lymph follicles. The infiltrating histiocytes showed emperipolesis. The histiocytes were immunoreactive for S-100 protein and CD68 and negative for CD1a, leading to the diagnosis of intracranial RDD. The postoperative course was uneventful without further therapy. The dizziness had not re-appeared and MRI demonstrated no recurrence of tumors for 7 months. Intracranial RDD shows a male predominance and occurs later in life than nodal RDD. The clinical manifestations and prognosis are variable depending on the location of the tumor and treatment. Most intracranial RDD have a benign course, but long-term follow-up is important, because recurrence has been observed