title: Orbital Langerhans cell histiocytosis in adults.
abstract: OBJECTIVE
  To report 3 patients with adult-onset orbital Langerhans cell histiocytosis (LCH).
  
  
  METHODS
  Retrospective interventional case reports.
  
  
  METHODS
  Three adult patients with orbital LCH.
  
  
  METHODS
  Case reports from 2 different clinics and review of the scientific literature.
  
  
  METHODS
  Clinical course and radiologic and histologic findings.
  
  
  RESULTS
  Three healthy patients, aged 20 to 36 (mean, 27) years, presented with biopsy-proven unilateral orbital LCH. The initial presenting complaints were mainly orbital pain and headache with or without orbital swelling and minimal effect on ocular movements. There was radiologic evidence of disease centered on the greater wing of sphenoid bone in all cases. Biopsy via lateral canthotomy was performed with LCH confirmed based on histologic and immunohistochemical findings. There was no evidence of disease elsewhere on systemic investigations. One patient had curettage and intralesional corticosteroids, 1 patient had a craniotomy for more complete resection of the lesion, and the third patient was referred for radiotherapy. All patients achieved complete resolution of symptoms and signs. None of the patients demonstrated any evidence of distant or recurrent disease at follow-up.
  
  
  CONCLUSIONS
  Orbital LCH in adults is a rare, usually unifocal, and relatively benign disease most commonly affecting the greater wing of sphenoid bone, where there is still active bone marrow in young adults. There is no consensus on cause or therapy, but our reported cases show that several treatment modalities are effective.
authors.affiliation: Royal Victorian Eye and Ear Hospital, Melbourne, Australia.
authors.forename: Ning
authors.initials: N
authors.lastname: Cheung
authors.forename: Dinesh
authors.initials: D
authors.lastname: Selva
authors.forename: Alan A
authors.initials: AA
authors.lastname: McNab
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000378:metabolism; D006646:Histiocytosis, Langerhans-Cell / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009916:Orbital Diseases / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D012189:Retrospective Studies; D013100:Sphenoid Bone / Q000000981:diagnostic imaging / Q000473:pathology; D014057:Tomography, X-Ray Computed
chemical_list: D015415:Biomarkers