title: Orbital schwannomatosis in the absence of neurofibromatosis.
abstract: OBJECTIVE
  The aim of this study was to describe 3 cases of primary orbital schwannomatosis without associated systemic neurofibromatosis.
  
  
  METHODS
  This is a retrospective interventional study of 3 patients who presented with multiple, distinct masses in the orbit (n = 3) as well as in the hemiface (n = 1). The clinical presentation, imaging features, surgical procedures, and outcomes were defined.
  
  
  RESULTS
  Two women and a man presented with of exophthalmos and diplopia. Pain was the most prominent complaint in 2 patients. None of the patients had associated systemic neurofibromatosis by history or examination. Radiologic evaluation with computed tomography or magnetic resonance imaging of orbit revealed multiple well-demarcated intraconal and extraconal masses. Masses were excised, and histopathology confirmed all masses to be schwannomas. Postoperative follow-up was uneventful with alleviation of primary complaints in all patients.
  
  
  CONCLUSIONS
  Multiple orbital schwannomas (primary orbital schwannomatosis) may be observed in patients without systemic association of neurofibromatosis. Management includes surgical excision of the tumors to achieve relief from their mass effects.
authors.affiliation: From the *Department of Ophthalmology, Selcuk University, Konya, Turkey; and †Department of Ophthalmology, University of California San Francisco, California.
authors.forename: Bengu Ekinci
authors.initials: BE
authors.lastname: Koktekir
authors.forename: H Jane
authors.initials: HJ
authors.lastname: Kim
authors.forename: Mike
authors.initials: M
authors.lastname: Geske
authors.forename: Michelle
authors.initials: M
authors.lastname: Bloomer
authors.forename: Reza
authors.initials: R
authors.lastname: Vagefi
authors.forename: Robert C
authors.initials: RC
authors.lastname: Kersten
mesh_terms: D000328:Adult; D004172:Diplopia / Q000175:diagnosis; D005094:Exophthalmos / Q000175:diagnosis; D005153:Facial Neoplasms / Q000175:diagnosis* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D008970:Molecular Weight; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis* / Q000601:surgery; D009442:Neurilemmoma / Q000175:diagnosis* / Q000601:surgery; D017253:Neurofibromatoses / Q000175:diagnosis* / Q000601:surgery; D009918:Orbital Neoplasms / Q000175:diagnosis* / Q000601:surgery; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000601:surgery; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome