• World Neurosurg · Jan 2016

    Review Case Reports

    Rosai-Dorfman Disease of rare isolated spinal involement: report of four cases and literature review.

    • Bo Yuan Huang, Hua Zhang, Wen Jing Zong, and Yan Hui Sun.
    • Department of Neurosurgery, San Bo Brain Hospital, Capital Medical University, Beijing, China.
    • World Neurosurg. 2016 Jan 1; 85: 367.e11-6.

    BackgroundRosai-Dorfman disease (RDD) is a rare histioproliferative disorder that only occasionally involves the central nervous system (CNS).Case DescriptionThe diagnosis and treatment of 4 patients with isolated spinal RDD are discussed. All 4 patients were treated by total or subtotal surgical resection and none of them experienced recurrence. Histopathologic examination showed a characteristic emperipolesis, and the lymphocytes were engulfed in the S-100-protein-positive histiocytes with no expression of CD1a.ConclusionsPreoperative diagnosis of spinal RDD is still challenging because the lesion is usually a dura-based lesion that mimics a meningioma. Surgical resection is an effective treatment, and radiotherapy, steroid therapy, and chemotherapy have not shown reliable therapeutic efficiency.Copyright © 2016 Elsevier Inc. All rights reserved.

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