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

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.

Medienart:

E-Artikel

Erscheinungsjahr:

2021

Erschienen:

2021

Enthalten in:

Zur Gesamtaufnahme - volume:10

Enthalten in:

Systematic reviews - 10(2021), 1 vom: 18. Jan., Seite 31

Sprache:

Englisch

Beteiligte Personen:

Hu, Pan-Pan [VerfasserIn]
Wei, Feng [VerfasserIn]
Liu, Xiao-Guang [VerfasserIn]
Liu, Zhong-Jun [VerfasserIn]

Links:

Volltext

Themen:

Clinical features
Journal Article
Literature review
Review
Rosai-Dorfman disease
Spine
Systematic Review
Treatment

Anmerkungen:

Date Completed 24.06.2021

Date Revised 24.06.2021

published: Electronic

Citation Status MEDLINE

doi:

10.1186/s13643-021-01581-0

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM320241432