Rare presentation of Rosai-Dorfman disease mimicking a cervical intramedullary spinal cord tumor.

BACKGROUND: Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is a rare, benign, lymphoproliferative disorder that commonly manifests as a massive painless cervical lymphadenopathy with associated fever and weight loss. Central nervous system involvement is extremely rare, and cervical spinal cord manifestation has not been reported. METHODS: A 76-year-old man presented with a 10-week history of right hemiparesis. Magnetic resonance imaging identified an enhancing cervical intramedullary lesion consistent with a primary spinal cord tumor. RESULTS: Histopathology revealed intramedullary histiocytosis. CONCLUSIONS: This cervical presentation of Rosai-Dorfman disease with central nervous system involvement suggests variation in the clinical manifestations of the disease, necessitating greater surgical awareness.