• Bo Yuan Huang, Hai Long Liu, and Chun Jiang Yu.
• Department of Neurosurgery, San Bo Brain Hospital, Capital Medical University, Beijing, China.
• World Neurosurg. 2016 Apr 1; 88: 694.e11-694.e15.

BackgroundRosai-Dorfman disease (RDD) is a rare histioproliferative disorder that only occasionally involves the central nervous system.Case DescriptionWe present the diagnosis and treatment of an exceedingly rare case of isolated intramedullary spinal RDD that has been reported only 3 times previously. Moreover, it is the first time that intramedullary spinal RDD has been described in a child. The patient was treated by total surgical resection and experienced no recurrence during the 12-month follow-up. Histopathologic examination showed a characteristic emperipolesis; the lymphocytes were engulfed in the S-100-protein-positive histiocytes with negative expression of CD1a.ConclusionsPreoperative diagnosis of spinal RDD is still challenging because the lesion usually is a dura-based lesion that mimics a meningioma. Surgical resection is an effective treatment and radiotherapy; and steroid and chemotherapy have not demonstrated reliable therapeutic efficiency.Copyright © 2016 Elsevier Inc. All rights reserved.

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