Characteristics of Rosai-Dorfman Disease Primarily Involved in the Central Nervous System: 3 Case Reports and Review of Literature

• Published in: World neurosurgery 2017-01, Vol.97, p.58-63
• Main Authors: Luo, Zhengxiang, Zhang, Yansong, Zhao, Penglai, Lu, Hucheng, Yang, Kun, Zhang, Yuhai, Zeng, Yanjun
• Format: Article
• Language: English
• Subjects: Adult; Aged; Central Nervous System - diagnostic imaging; Central Nervous System - drug effects; Central Nervous System - radiation effects; Central Nervous System - surgery; CNS; Female; Histiocytosis, Sinus - diagnostic imaging; Histiocytosis, Sinus - pathology; Histiocytosis, Sinus - therapy; Humans; Longitudinal Studies; Magnetic Resonance Imaging; Male; Neurosurgical Procedures - methods; Operation; Radiotherapy; Radiotherapy - methods; Rosai-Dorfman disease; Steroid; Steroids - therapeutic use; Tomography Scanners, X-Ray Computed
• ISSN: 1878-8750; 1878-8769
• DOI: 10.1016/j.wneu.2016.09.084

This study aimed to summarize the clinical characteristics of Rosai-Dorfman disease primarily involving the central nervous system and to explore diagnosis and treatment. 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. 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. 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.