Rosai-Dorfman Disease of Rare Isolated Spinal Involvement: Report of 4 Cases and Literature Review

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• 作者：Bo Yuan Huang¹ ; Hua Zhang² ; ^{zhanghua3579@hotmail.com" class="auth_mail" title="E-mail the corresponding author} ; Wen Jing Zong³ ; Yan Hui Sun² ; ^{525111209@qq.com" class="auth_mail" title="E-mail the corresponding author}
• 关键词：Isolated spinal involvement ; Rosai-Dorfman disease
• 刊名：World Neurosurgery
• 出版年：2016
• 出版时间：January 2016
• 年：2016
• 卷：85
• 期：Complete
• 页码：367.e11-367.e16
• 全文大小：1432 K

文摘

Rosai-Dorfman disease (RDD) is a rare histioproliferative disorder that only occasionally involves the central nervous system (CNS).

Case Description

The 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.

Conclusions

Preoperative 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.