Surgical Treatment of Intracranial Rosai-Dorfman Disease

Rosai-Dorfman disease (RDD) is a rare non-Langerhans histiocytosis characterized by systemic lymphadenopathy and extranodal involvement. Isolated central nervous system (CNS) involvement accounts for <5% of cases. Its clinical and radiological features often mimic meningioma, lymphoma, or metastatic disease, leading to frequent misdiagnosis. Treatment outcomes remain unpredictable. Here, we report a case of extensive intracranial RDD managed with surgical resection and corticosteroid therapy. Combined with a literature review, we summarize the radiological-pathological characteristics of CNS-RDD and propose that gross total resection (GTR) combined with steroid therapy is effective in reducing perilesional edema and improving clinical outcomes.