Intracranial multifocal Rosai-Dorfman disease

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is an uncommon benign histioproliferative disorder with systemic symptoms and lymphadenopathy. Mostly affecting lymph nodes, RDD typically presents as substantial painless bilateral cervical lymphadenopathy associated with fever, leukocytosis, anemia, polyclonal hypergammaglobulinemia, and increased erythrocyte sedimentation rate. Extranodal manifestations occur in approximately 43% of patients. Common extranodal sites include skin, respiratory tract, orbit, paranasal sinuses, soft tissue and bone, whereas CNS involvement is considered extremely rare. Herein, we describe a unique case of intracranial RDD presenting with multifocal lesions.