Objective: Rosai-Dorfman disease (RDD) is a rare idiopathic, non-neoplastic histioproliferative disease. Central nervous system (CNS) manifestations are extremely rare. In this paper, we describe a 6-year-old boy with intracranial RDD mimicking multiple meningiomas both clinically and radiologically. We reviewed the literature to understand the clinical behaviour, clinicopathological features and treatment options.
Methods: A PubMed (US National Library of Medicine) search using the keywords 'Rosai-Dorfman disease' and 'central nervous system' was performed and citations were reviewed.
Results: Eighty-five cases of RDD involving the CNS have been reported until date, and only 7 cases involved children. Of the 85 cases, 16 cases mimicked multiple meningiomas. Our case is the first to involve multiple lesions in a child under 14 years old.
Conclusion: After reviewing the literature, we concluded that RDD should be considered as a differential diagnosis for lesions mimicking multiple meningiomas, especially in children. Resection of the intracranial lesion is the most effective treatment, and a definitive diagnosis should be based on histopathologic and immunocytochemical examinations.