Rosai-Dorfman-Destombes disease of the nervous system: a systematic literature review.

ABSTRACT:

Background

Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytic disorder with heterogeneous clinical manifestations and rare neurologic involvement. The existing clinical literature about neurologic RDD has yet to be critically examined.

Methods

We performed a four-database English-language systematic literature search for cases of RDD neurohistiocytosis, excluding secondary literature. Individual patient data for neurologic symptoms, disease sites, treatments, and responses were captured. Responses to first-line and second-line surgical interventions, post-surgical radiotherapy, and systemic therapies were analyzed.

Results

Among 4769 articles yielded by literature search, 154 articles were fully reviewed, containing data on 224 patients with neurologic RDD. 1

SUBMITTER: Nasany RA 

PROVIDER: S-EPMC8889645 | biostudies-literature | 2022 Mar

REPOSITORIES: biostudies-literature