Systemic Rosai-Dorfman disease with central nervous system involvement

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.