Issue release date: 12.11.2024
Turkish Neurosurgery
2015 , Vol 25 , Num 1
Isolated Intracranial Rosai-Dorfman Disease Involving the Meninges: Report of a Rare Case
Sapthagiri Institute of Medical Sciences and Research Center, Department of Pathology, Karnataka, India
DOI :
10.5137/1019-5149.JTN.10051-13.0
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