title: Primary orbital intraosseous hemangioma.
abstract: OBJECTIVE
  Primary orbital intraosseous hemangioma represents a rare, histopathologically benign, vascular tumor of the bony orbit. Only 41 cases have been documented in the literature to date. The authors present 4 new cases of the disease and review the relevant literature.
  
  
  METHODS
  Retrospective, multicenter case note analysis of 4 patients with histopathologically confirmed primary orbital intraosseous hemangioma and a systematic review of the English-language literature.
  
  
  RESULTS
  Four new cases of cavernous haemangiomata are presented with varying clinical manifestations, radiologic appearances, and treatments. Literature review (including the present 4 cases) yielded 45 cases in total. Presentation is often in the fourth and fifth decades (42% cases), the frontal bone being most commonly affected, followed by the zygoma, sphenoid, and maxilla. Intracranial extension occurred in 4 cases. Median duration of symptoms before presentation was 12 months (range, 1 month to 15 years) and the most frequent presentation was a painless mass, often on the orbital rim. The radiologic findings are reviewed. Histopathologically, the lesions were cavernous in 80%, capillary in 17%, and mixed in 3%; the capillary subtype seemed to be associated with more aggressive disease. Treatment was mainly by surgical excision and occasionally complicated by significant blood loss; preoperative embolization of lesions may reduce bleeding.
  
  
  CONCLUSIONS
  Primary orbital intraosseous hemangioma is a rare vascular tumor that typically presents with a mass effect in the orbits of patients in the fourth and fifth decades of life. Preoperatively, it is important to be cognizant of the possible diagnosis as surgery can be complicated by life-threatening hemorrhage.
authors.affiliation: Discipline of Ophthalmology and Visual Sciences, University of Adelaide, Adelaide, South Australia. drsimonmadge@gmail.com
authors.forename: Simon N
authors.initials: SN
authors.lastname: Madge
authors.forename: Sumu
authors.initials: S
authors.lastname: Simon
authors.forename: Zurina
authors.initials: Z
authors.lastname: Abidin
authors.forename: Raf
authors.initials: R
authors.lastname: Ghabrial
authors.forename: Garry
authors.initials: G
authors.lastname: Davis
authors.forename: Alan
authors.initials: A
authors.lastname: McNab
authors.forename: Dinesh
authors.initials: D
authors.lastname: Selva
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D018324:Hemangioma, Capillary / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D006392:Hemangioma, Cavernous / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed