Intracranial Rosai-Dorfman Disease: A Case to Remember

Sinus histiocytosis with massive lymphadenopathy (SHML), an alternative term for Rosai-Dorfman disease (RDD), is a rare benign idiopathic immune-related lymphoproliferative condition. The central nervous system (CNS) has been documented to be involved in RDD, although lymph nodes are the organs that are most frequently and primarily associated with the disease manifestation. Nonetheless, CNS involvement in RDD is rare and poorly understood. As a result, there is a lack of a solid basis for therapeutic approaches for CNS involvement in RDD. Here, we present a case of RDD with cerebral involvement, a rare presentation of RDD with atypical symptoms. A brief assessment of the radiographic appearance, histological findings, and the peculiar manifestations of the disease is provided.