Isolated Intracranial Rosai-Dorfman Disease: A Diagnostic Challenge
Abstract
Preeti Singh, Harsh Patel and Abhaya Kumar
Isolated intracranial Rosai-Dorfman disease (RDD) is an extremely rare, idiopathic histo-proliferative disorder. RDD is associated with the proliferation of histiocytes and emperipolesis. Central nervous system (CNS) involvement is extremely rare and due to rarity of RDD, it is not usually proposed in intracranial lesions diagnosis. RDD radiologically mimics meningioma and dural metastasis as dural-based lesions and histologically mimics plasma cell granuloma, Langerhans cell histiocytosis (LCH) and lymphoproliferative disease. We report a case with isolated intracranial RDD. A 47-year- old man presented with focal seizure. This case preoperatively was misdiagnosed with meningioma. Histopathological examination revealed pale histiocytes displaying emperipolesis which were positive for S-100 and CD68 proteins and negative for CD1a marker. BRAF V600E mutation was negative. In this case, total resection was performed and clinical symptoms were regressed completely.