Isolated intracranial Rosai-Dorfman disease: case report and literature review

Pathol Int. 1998 May;48(5):396-402. doi: 10.1111/j.1440-1827.1998.tb03923.x.


Rosai-Dorfman disease, first described in 1969, is a rare idiopathic histioproliferative disease affecting the lymph nodes. Typical clinical features include bilateral painless lymphadenopathy, fever and polyclonal hypergammaglobulinemia. In approximately 43% of cases, extranodal sites may be involved and occasionally represent the initial or sole manifestation of the disease. Central nervous system manifestations are exceedingly rare, and only 17 cases have been recorded, among which there are merely seven isolated intracranial cases without concurrent nodal or other extranodal involvement. Herein, we report a 38-year-old male presenting with generalized tonic-clonic seizure and radiological findings indicative of meningioma. Complete physical examinations and laboratory surveys demonstrate the absence of involvement at other body sites. Microscopically, the lesion consists of proliferative histiocytes exhibiting emperipolesis coupled with the characteristic cytoplasmic staining against S-100 protein. The differential diagnosis of polymorphic inflammatory meningioma-mimicking masses is discussed, and a review of previously reported intracranial Rosai-Dorfman disease is presented.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Brain Diseases / diagnosis*
  • Brain Diseases / metabolism
  • Diagnosis, Differential
  • Histiocytosis, Sinus / diagnosis*
  • Histiocytosis, Sinus / metabolism
  • Humans
  • Immunohistochemistry
  • Male
  • Microscopy, Electron
  • S100 Proteins / metabolism
  • Tomography, X-Ray Computed


  • S100 Proteins