Giant intracranial Rosai-Dorfman disease

Ture U., Seker A., BOZKURT S., Uneri C., Sav A., Pamir M.

JOURNAL OF CLINICAL NEUROSCIENCE, vol.11, no.5, pp.563-566, 2004 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Volume: 11 Issue: 5
  • Publication Date: 2004
  • Doi Number: 10.1016/j.jocn.2003.11.012
  • Journal Name: JOURNAL OF CLINICAL NEUROSCIENCE
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.563-566
  • Keywords: cavernous sinus, cranio-orbitozygomatic approach, lateral rhinotomy, Rosai-Dorfman disease, sinus histiocytosis, EXTRANODAL SINUS HISTIOCYTOSIS, CENTRAL-NERVOUS-SYSTEM, MASSIVE LYMPHADENOPATHY, INVOLVEMENT, MENINGIOMA, MANIFESTATIONS, ENTITY, MASSES
  • Ondokuz Mayıs University Affiliated: No

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. (C) 2003 Elsevier Ltd. All rights reserved.