Hu Pan-Pan, Wei Feng, Liu Xiao-Guang, Liu Zhong-Jun
Department of Orthopaedics and Beijing Key Laboratory of Spinal Disease Research, Peking University Third Hospital, No. 49 North Garden Rd, Haidian District, Beijing, 100191, China.
Syst Rev. 2021 Jan 18;10(1):31. doi: 10.1186/s13643-021-01581-0.
To review and summarize the clinical features, diagnosis, treatment strategies, and prognosis of spinal Rosai-Dorfman disease (RDD).
RDD is also termed as sinus histiocytosis with massive lymphadenopathy. We searched the databases of PubMed, Elsevier ScienceDirect, SpringerLink, and OVID. The keywords were Rosai-Dorfman disease and spine/central nervous system. Research articles and case reports with accessibility to full texts regarding spinal RDD were eligible for the inclusion. A total of 62 articles were included, and they contained 69 cases. We extracted the information of interest and analyzed them using SPSS statistics package.
The average age was 33.1 ± 18.3 years. The ratio of males to females was 1.9/1. Overall, 63 cases presented with spine-related symptoms. A total of 27 cases (39.1%) had multi-organ lesions, and 12 cases had records of massive lymphadenopathy. Among 47 cases who first manifested spine-related symptoms, 93.6% were preoperatively misdiagnosed. The disease had a predilection for cervical spine (38.8%) and thoracic spine (40.3%). 62.9% of lesions were dura-based. Surgery remained the mainstream treatment option (78.8%), with or without adjuvant therapies. Total lesion resection was achieved in 34.8% of cases. The rate of lesion recurrence/progression was 19.5%, which was marginally lower for total resection than for non-total resection.
Spinal RDD has no pathognomonic clinical and imaging features. Most cases first present with spine-relevant symptoms. Massive lymphadenopathy is not common, but a tendency for multi-organ involvement should be considered. Spinal RDD has a high recurrence rate; thus, total resection is the treatment of choice. Adjuvant therapies are indicated for multi-organ lesions and residual lesions. A wait and watch strategy is recommended for asymptomatic patients. Herein, a workflow of diagnosis and treatment of the spinal RDD is established.
回顾并总结脊柱Rosai-Dorfman病（RDD）的临床特征、诊断、治疗策略及预后。
RDD也被称为伴有巨大淋巴结病的窦性组织细胞增多症。我们检索了PubMed、爱思唯尔ScienceDirect、施普林格Link和OVID数据库。关键词为Rosai-Dorfman病和脊柱/中枢神经系统。可获取全文的关于脊柱RDD的研究文章和病例报告符合纳入标准。共纳入62篇文章，包含69例病例。我们提取了感兴趣的信息，并使用SPSS统计软件包进行分析。
平均年龄为33.1±18.3岁。男女比例为1.9/1。总体而言，63例出现与脊柱相关的症状。共有27例（39.1%）有多器官病变，12例有巨大淋巴结病记录。在最初表现出与脊柱相关症状的47例中，93.6%术前被误诊。该疾病好发于颈椎（38.8%）和胸椎（40.3%）。62.9%的病变以硬脑膜为基底。手术仍然是主要的治疗选择（78.8%），可联合或不联合辅助治疗。34.8%的病例实现了病变全切。病变复发/进展率为19.5%，全切病例的该比率略低于非全切病例。
脊柱RDD没有特征性的临床和影像学表现。大多数病例最初表现为与脊柱相关的症状。巨大淋巴结病并不常见，但应考虑多器官受累的倾向。脊柱RDD复发率高；因此，全切是首选治疗方法。多器官病变和残留病变需行辅助治疗。对于无症状患者，建议采取观察等待策略。在此，建立了脊柱RDD的诊断和治疗流程。
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