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Systematic Reviews
Published in: Systematic Reviews 1/2021

Open Access 01-12-2021 | Systematic review update

Diagnosis and treatment of Rosai-Dorfman disease of the spine: a systematic literature review

Authors: Pan-pan Hu, Feng Wei, Xiao-guang Liu, Zhong-jun Liu

Published in: Systematic Reviews | Issue 1/2021

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Abstract

Purpose

To review and summarize the clinical features, diagnosis, treatment strategies, and prognosis of spinal Rosai-Dorfman disease (RDD).

Methods

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.

Results

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.

Conclusion

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.
Appendix
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Metadata
Title
Diagnosis and treatment of Rosai-Dorfman disease of the spine: a systematic literature review
Authors
Pan-pan Hu
Feng Wei
Xiao-guang Liu
Zhong-jun Liu
Publication date
01-12-2021
Publisher
BioMed Central
Published in
Systematic Reviews / Issue 1/2021
Electronic ISSN: 2046-4053
DOI
https://doi.org/10.1186/s13643-021-01581-0

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