Central Nervous System Manifestations of Rosai-Dorfman Disease: A Case Series

Rosai-Dorfman disease (RDD) with central nervous system (CNS) involvement poses substantial diagnostic challenges due to its propensity to mimic meningiomas or inflammatory pathologies. We present a series of three RDD cases with CNS involvement to evaluate their clinical, radiological, and histopathological features. The series includes a 30-year-old female with pachymeningitis and spinal involvement; a 66-year-old male with a skull base lesion; and a 45-year-old male presenting with trigeminal neuralgia secondary to a cavernous sinus lesion. In all instances, definitive diagnosis was achieved following surgical intervention and histopathological analysis, revealing hallmark emperipolesis and a characteristic immunohistochemical profile (S100+, CD68+, and CD1a-). Management comprised neurosurgical resection for decompression and diagnosis, supplemented by adjuvant corticosteroids and chemotherapy where indicated. This series underscores that RDD is a critical differential diagnosis for atypical extra-axial CNS lesions. A multidisciplinary approach is essential for optimizing patient outcomes.