title: Adult orbital xanthogranulomas: clinical features and management.
abstract: OBJECTIVE
  Adult-onset asthma with periocular xanthogranuloma and adult-onset xanthogranuloma are 2 rare subtypes of non-Langerhans cell histiocytic disorder and much remains unknown regarding optimal treatment. The authors describe their experience in the management of these 2 disease subtypes.
  
  
  METHODS
  This is a retrospective case series with histopathologically proven orbital xanthogranuloma over a period of 12 years. Clinical, imaging, and histopathologic features; associated systemic conditions; treatment modality; and outcome during follow-up of 6 adult patients who had adult-onset asthma with periocular xanthogranuloma and adult-onset xanthogranuloma were reviewed.
  
  
  RESULTS
  The age range was 29-75 years (median 56 years). The duration of symptoms and signs varied from 10 months to 9 years. All patients had bilateral and asymmetric involvement. Palpebral swelling with yellow discoloration and upper eyelid ptosis were the most common signs. Adult-onset asthma was present in 2 patients. Imaging studies demonstrated ill-defined infiltrative lesions involving the preseptal area, lacrimal glands, extraocular muscles, retrobulbar fat, and optic nerves. The median follow-up was 50 months. Complete regression of all clinical signs was obtained at 8 months, whereas imaging findings disappeared at 18 months with treatment. No recurrence was observed.
  
  
  CONCLUSIONS
  Treatment consisting of debulking as much affected soft tissue as possible followed by a 3-day course of intravenous pulse methylprednisolone administration and then by oral prednisone for at least 6 months may provide adequate regression of the granulomas without recurrence and satisfactory cosmesis in patients with adult orbital xanthogranuloma with and without asthma.
authors.affiliation: 1 Ocular Oncology Service, Department of Ophthalmology, Hacettepe University School of Medicine, Ankara - Turkey.
authors.forename: Hayyam
authors.initials: H
authors.lastname: Kiratli
authors.forename: Meltem
authors.initials: M
authors.lastname: Kiliç
authors.forename: Berçin
authors.initials: B
authors.lastname: Tarlan
authors.forename: Figen
authors.initials: F
authors.lastname: Söylemezoglu
mesh_terms: D000284:Administration, Oral; D000328:Adult; D000368:Aged; D001249:Asthma / Q000175:diagnosis / Q000188:drug therapy; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006099:Granuloma / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D009916:Orbital Diseases / Q000175:diagnosis* / Q000188:drug therapy; D011241:Prednisone / Q000627:therapeutic use; D020551:Pulse Therapy, Drug; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014973:Xanthomatosis / Q000175:diagnosis* / Q000188:drug therapy
chemical_list: D005938:Glucocorticoids; D011241:Prednisone; D008775:Methylprednisolone