Primary intracerebral Rosai-Dorfman disease.
Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), is an idiopathic histiocytic disorder of lymph nodes and extranodal sites. Central nervous system (CNS) manifestations, particularly in the absence of nodal disease, are rare. Intracranial RDD clinically and radiologically resembles meningioma, and histologic examination is essential for a definitive diagnosis. We report four patients with RDD primary to the CNS without evidence of other sites of involvement, review the literature, and discuss the clinical manifestations, pathology, treatment and outcome.
Zhang
Jia-Tang
JT
Department of Neurology, The Chinese People's Liberation Army (PLA) General Hospital, 28 Fuxing Road, Hai Dian District, Beijing 100853, China. thj96236@163.com
Tian
Hui-Jun
HJ
Lang
Sen-Yang
SY
Wang
Xiang-Qin
XQ
D000328:Adult; D001921:Brain / Q000473:pathology*; D005260:Female; D015618:Histiocytosis, Sinus / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male