• Ziyi Li, Chuan Zhou, Ge Chen, and Yuhai Bao.
• Department of Neurosurgery, The Second Affiliated Hospital of Nanchang University, Nanchang, China.
• World Neurosurg. 2018 Nov 1; 119: 249-255.

BackgroundRosai-Dorfman disease (RDD), also called sinus histiocytosis with massive lymphadenopathy, is an idiopathic, non-neoplastic, lymphoproliferative disorder. Histologically, the disease is characterized by emperipolesis and S-100 immunoreactivity. RDD usually presents with massive painless cervical lymphadenopathy, with only 5% of cases affecting the central nervous system.Case DescriptionWe report the case of a 46-year-old woman with isolated RDD of the left cavernous sinus with left facial numbness and weakness of the left masticatory muscle. The patient was successfully treated by total resection without recurrence found at the 65-month follow-up examination.ConclusionsCentral nervous system RDD is a diagnostic challenge preoperatively depending on the radiologic findings. Immunohistochemical confirmation is indispensable for the definite diagnosis. Total resection remains the most effective treatment to date. Adjuvant treatment such as chemotherapy, radiotherapy, and steroids can be administrated in cases of incomplete resection, recurrence, or multiple foci.Copyright © 2018 Elsevier Inc. All rights reserved.

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