Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease (RDD) is a reactive condition of unknown etiology, characterised by a proliferation of histiocytes exhibiting emperipolesis of lymphocytes and plasma cells. It usually presents as bilateral painless cervical lymphadenopathy. Extranodal RDD without nodal disease is seen in 23% of the cases. Intracranial RDD occurs in less than 5% of the total number of patients with extranodal disease. Isolated intracranial RDD in the absence of nodal disease is exceptional.We report a case of Rosai-Dorfman disease with isolated intracranial involvement. A 38-year-old man came with long-standing headache and the magnetic resonance imaging (MRI) scan showed features of meningioma. The histopathology revealed sheets of histiocytes displaying emperipolesis. These histiocytes were S100 positive but CD1a and epithelial membrane antigen negative.KEywOrds: Emperipolesis, Extranodal Rosai-Dorfman disease, Intracranial Rosai-Dorfman disease, Sinus histiocytosis with massive lymphadenopathy ÖZMasif lenfadenopatili sinüs histiyositozu veya Rosai-Dorfman hastalığı (RDD) bilinmeyen etiyolojili reaktif bir durumdur ve lenfositler ve plazma hücrelerinin emperipolezisini gösteren bir histiosit proliferasyonuyla karakterizedir. Genellikle bilateral ağrısız servikal lenfadenopati olarak ortaya çıkar. Nodal hastalık olmadan ekstranodal RDD olguların %23'ünde görülür. İntrakraniyal RDD, ekstranodal hastalıklı hastaların %5'inden azında görülür. Nodal hastalık bulunmadığında izole intrakraniyal RDD çok nadirdir.İzole intrakraniyal tutulumlu bir Rosai-Dorfman hastalığı olgusu sunuyoruz. 38 yaşında bir erkek, uzun süreli baş ağrısı ile başvurdu ve manyetik rezonans görüntüleme (MRG) taraması menenjiyom bulguları gösterdi. Histopatolojide emperipolez gösteren histiyosit tabakaları görüldü. Bu histiyositler S100 pozitif ama CD1a ve epitelyal membran antijeni negatifti.ANAhTAr sÖZCÜKlEr: Emperipolez, Ekstranodal Rosai-Dorfman hastalığı, İntrakraniyal Rosai-Dorfman hastalığı, Masif lenfadenopatili sinüs histiyositozu
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