title: Diagnosis and management of Rosai-Dorfman disease involving the central nervous system.
abstract: BACKGROUND
  Rosai-Dorfman disease is a benign non-neoplastic proliferative disorder of histiocytes originally described in the cervical lymph nodes. Extranodal sites were later recognized, and by 1990, they were shown to represent over 40% of cases; however, central nervous system involvement is still considered rare. We review the literature, which shows a steady increase in reports of Rosai-Dorfman disease involving the brain and/or spine.
  
  
  METHODS
  A literature search was performed for the period 1969-2008, using multiple search engines, with keywords Rosai-Dorfman disease, central nervous system Rosai-Dorfman disease and sinus histiocytosis with massive lymphadenopathy.
  
  
  RESULTS
  By December 2008, 111 cases of Rosai-Dorfman disease involving the central nervous system had been reported in the literature, including our cases. In the central nervous system, Rosai-Dorfman disease is ubiquitous. Although it is characterized by unique, indeed pathognomonic, histopathological cytoarchitecture, it may be mistaken for many other neoplastic and inflammatory histioproliferative diseases. Surgical resection with post-operative corticosteroids remains the treatment of choice.
  
  
  CONCLUSIONS
  Rosai-Dorfman disease involving the central nervous system appears to be more common than previously thought and may well assume a more prominent place in the differential diagnoses of dural-based lesions. Expert awareness of the characteristic histopathology and immunohistochemistry of the disease is required for accurate diagnosis.
authors.affiliation: Division of Neurological Surgery, Department of Surgery, College of Medicine, University of Ibadan and University College Hospital, Ibadan, Nigeria.
authors.forename: Amos O
authors.initials: AO
authors.lastname: Adeleye
authors.forename: Gail
authors.initials: G
authors.lastname: Amir
authors.forename: Shifra
authors.initials: S
authors.lastname: Fraifeld
authors.forename: Yigal
authors.initials: Y
authors.lastname: Shoshan
authors.forename: Felix
authors.initials: F
authors.lastname: Umansky
authors.forename: Sergey
authors.initials: S
authors.lastname: Spektor
mesh_terms: D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D016208:Databases, Factual / Q000706:statistics & numerical data; D015618:Histiocytosis, Sinus / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D012189:Retrospective Studies