ABSTRACT
Rosai–Dorfman disease (RDD) is a rare idiopathic and lymphoproliferative disorder. Central nervous system (CNS) involvement is infrequent and typically manifests as an isolated lesion. In this article, we describe an unusual case of RDD with multiple lesions in the CNS, upper respiratory tract and lymph nodes. A literature review revealed 45 cases (including the one described herein) of systemic RDD with CNS involvement documented to date. Among these cases, 29 (64.4%) presented with intracranial lesions, 10 (22.2%) with spinal lesions and 6 (13.3%) with both. While the condition of most patients was stable, only four died from RDD or for other reasons. Prognosis is good for most patients, even those with extensive lesions. Although there are various treatments for RDD, surgery is preferred. Given the rarity of RDD, multicenter international collaborations are advocated in order to study disease pathogenesis and develop effective treatment strategies.
Disclosure statement
The authors report no conflicts of interest.
Additional information
Notes on contributors
Song Tan
Song Tan is a surgeon of Department of Neurosurgery, the first affiliated hospital of Chongqing Medical University.
Lunliang Ruan
Lunliang Ruan is a surgeon of Department of Neurosurgery, the first affiliated hospital of Chongqing Medical University.
Kai Jin
Kai Jin is a surgeon of Department of Neurosurgery, the first affiliated hospital of Chongqing Medical University.
Fuchao Wang
Fuchao Wang is a surgeon of Department of Neurosurgery, the first affiliated hospital of Chongqing Medical University.
Jiamin Mou
Jiamin Mou is a surgeon of Department of Neurosurgery, the first affiliated hospital of Chongqing Medical University.
Hua Huang
Hua Huang is a surgeon of Department of Neurosurgery, the first affiliated hospital of Chongqing Medical University.
Gang Yang
Gang Yang is a professor at Chongqing Medical University and a surgeon of Department of Neurosurgery, the first affiliated hospital of Chongqing Medical University.