Giant intracranial Rosai-Dorfman disease

J Clin Neurosci. 2004 Jun;11(5):563-6. doi: 10.1016/j.jocn.2003.11.012.

Abstract

Rosai-Dorfman disease is a rare, non-neoplastic disease characterized by an unusual proliferation of histiocytic cells. It rarely manifests intracranially, and only 50 cases of intracranial lesions have been reported. We describe an unusual case of a huge, solid mass in the paranasal sinuses, orbits, cavernous sinuses, and suprasellar cisterns. A 29-year-old patient was admitted to our hospital with nasal obstruction and proptosis and visual loss in the right eye. A biopsy was done at another institution and the diagnosis was "pseudotumor of orbit". A right-sided cranio-orbitozygomatic craniotomy combined with a right-sided lateral rhinotomy was used to excise the tumor with right orbital exenteration. The histopathological diagnosis was consistent with Rosai-Dorfman disease. The patient underwent postoperative chemotherapy. Involvement of the central nervous system in Rosai-Dorfman disease is rare, but the disease's ability to mimic other pathologies underlines its importance.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Craniotomy / methods
  • Histiocytosis, Sinus / pathology*
  • Histiocytosis, Sinus / surgery
  • Humans
  • Magnetic Resonance Imaging / methods
  • Male
  • Paranasal Sinuses / pathology*
  • Paranasal Sinuses / surgery
  • Tomography, X-Ray Computed / methods