Characteristics of Rosai-Dorfman Disease Primarily Involved in the Central Nervous System: 3 Case Reports and Review of Literature.
OBJECTIVE
This study aimed to summarize the clinical characteristics of Rosai-Dorfman disease primarily involving the central nervous system and to explore diagnosis and treatment.


METHODS
We analyzed the clinical, imaging, and pathologic characteristics; treatment; and prognosis in 3 cases of Rosai-Dorfman disease primarily involving the central nervous system. We also performed a literature review.


RESULTS
The largest of multiple intracranial lesions was totally resected, and steroid administration and radiotherapy were performed in phases for the remaining lesions. During the 1-year follow-up period, the excised lesion did not recur, and no obvious variations were observed in the other lesions. Subtotal resection was performed of the largest of another group of multiple intracranial lesions, and the residual did not show any obvious variations during the 1-year follow-up period. The isolated lesion was totally resected and did not recur during a 2-year follow-up period.


CONCLUSIONS
Rosai-Dorfman disease with multiple lesions primarily involving the central nervous system is rare. Imaging characteristics are similar to meningiomas, and the pathological features include lymphocytes and plasma cells reaching tissue cells with large volume and abundant cytoplasm. Surgery is the preferred treatment, as the effects of steroid administration and radiotherapy are not apparent.
Department of Neurosurgery, NanJing Brain Hospital affiliated with NanJing Medical University, NanJing, China. Electronic address: 342766863@qq.com.
Zhengxiang
Z
Luo
Department of Neurosurgery, NanJing Brain Hospital affiliated with NanJing Medical University, NanJing, China.
Yansong
Y
Zhang
Department of Neurosurgery, NanJing Brain Hospital affiliated with NanJing Medical University, NanJing, China.
Penglai
P
Zhao
Department of Neurosurgery, NanJing Brain Hospital affiliated with NanJing Medical University, NanJing, China.
Hucheng
H
Lu
Department of Neurosurgery, NanJing Brain Hospital affiliated with NanJing Medical University, NanJing, China.
Kun
K
Yang
Department of Neurosurgery, NanJing Brain Hospital affiliated with NanJing Medical University, NanJing, China.
Yuhai
Y
Zhang
Biomechanics and Medical Information Institute, Beijing University of Technology, Beijing, China. Electronic address: yjzeng@bjut.edu.cn.
Yanjun
Y
Zeng
D000328:Adult; D000368:Aged; D002490:Central Nervous System / Q000000981:diagnostic imaging* / Q000187:drug effects / Q000528:radiation effects / Q000601:surgery; D005260:Female; D015618:Histiocytosis, Sinus / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D019635:Neurosurgical Procedures / Q000379:methods*; D011878:Radiotherapy / Q000379:methods; D013256:Steroids / Q000627:therapeutic use; D015898:Tomography Scanners, X-Ray Computed
D013256:Steroids
CNS; Operation; Radiotherapy; Rosai-Dorfman disease; Steroid