Diagnosis and management of Rosai-Dorfman disease involving the central nervous system.
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.
Adeleye
Amos O
AO
Division of Neurological Surgery, Department of Surgery, College of Medicine, University of Ibadan and University College Hospital, Ibadan, Nigeria.
Amir
Gail
G
Fraifeld
Shifra
S
Shoshan
Yigal
Y
Umansky
Felix
F
Spektor
Sergey
S
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