TY - JOUR
T1 - Regression of intracranial Rosai-Dorfman disease following corticosteroid therapy
T2 - Case report
AU - McPherson, Christopher M.
AU - Brown, Justin
AU - Kim, Angela W.
AU - DeMonte, Franco
PY - 2006/5
Y1 - 2006/5
N2 - Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder usually presenting with massive, painless lymphadenopathy. Extranodal involvement has been reported including at least 50 cases affecting the central nervous system (CNS). The treatment of CNS RDD as reported in the literature has primarily involved a surgical technique. The authors report on the case of a 53-year-old man presenting with multiple skull base lesions mimicking meningiomas. The patient suffered visual deterioration and underwent a right orbitopterional craniotomy as well as optic nerve decompression. Histopathological analysis revealed histiocytic cells and emperipolesis consistent with RDD. Following surgery, corticosteroid agents were administered, leading to marked resolution of both the remaining surgically untreated lesions and the balance of the patient's symptoms. This report represents the first case of the resolution of intracranial RDD following corticosteroid therapy. Corticosteroid agents should be considered an effective option in the treatment of CNS RDD.
AB - Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder usually presenting with massive, painless lymphadenopathy. Extranodal involvement has been reported including at least 50 cases affecting the central nervous system (CNS). The treatment of CNS RDD as reported in the literature has primarily involved a surgical technique. The authors report on the case of a 53-year-old man presenting with multiple skull base lesions mimicking meningiomas. The patient suffered visual deterioration and underwent a right orbitopterional craniotomy as well as optic nerve decompression. Histopathological analysis revealed histiocytic cells and emperipolesis consistent with RDD. Following surgery, corticosteroid agents were administered, leading to marked resolution of both the remaining surgically untreated lesions and the balance of the patient's symptoms. This report represents the first case of the resolution of intracranial RDD following corticosteroid therapy. Corticosteroid agents should be considered an effective option in the treatment of CNS RDD.
KW - Corticosteroid therapy
KW - Multiple meningiomas
KW - Rosai-Dorfman disease
KW - Sinus histiocytosis
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U2 - 10.3171/jns.2006.104.5.840
DO - 10.3171/jns.2006.104.5.840
M3 - Article
C2 - 16703894
AN - SCOPUS:33646481626
SN - 0022-3085
VL - 104
SP - 840
EP - 844
JO - Journal of neurosurgery
JF - Journal of neurosurgery
IS - 5
ER -