Systemic Rosai–Dorfman disease with central nervous system involvement

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.

KEYWORDS:

• Sinus histiocytosis with massive lymphadenopathy
• Rosai–Dorfman disease
• central nervous system

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.