罕见孤立性脊柱受累的罗萨伊-多夫曼病：4例报告及文献复习
Rosai-Dorfman Disease of Rare Isolated Spinal Involvement: Report of 4 Cases and Literature Review.
作者信息
Huang Bo Yuan, Zhang Hua, Zong Wen Jing, Sun Yan Hui
机构信息
Department of Neurosurgery, San Bo Brain Hospital, Capital Medical University, Beijing, China.
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
出版信息
World Neurosurg. 2016 Jan;85:367.e11-6. doi: 10.1016/j.wneu.2015.09.097. Epub 2015 Oct 13.
BACKGROUND
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.
背景
罗萨伊-多夫曼病（RDD）是一种罕见的组织细胞增生性疾病，仅偶尔累及中枢神经系统（CNS）。
病例描述
讨论了4例孤立性脊柱RDD患者的诊断和治疗。所有4例患者均接受了全切除或次全切除手术，无一例复发。组织病理学检查显示特征性的血细胞吞噬现象，淋巴细胞被S-100蛋白阳性组织细胞吞噬，且无CD1a表达。
结论
脊柱RDD的术前诊断仍然具有挑战性，因为病变通常是基于硬脑膜的病变，类似脑膜瘤。手术切除是一种有效的治疗方法，而放疗、类固醇治疗和化疗尚未显示出可靠的治疗效果。
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