title: A clinical, radiologic, and immunopathologic study of five periorbital intraosseous cavernous vascular malformations.
abstract: OBJECTIVE
  To correlate the clinical, radiographic, histopathologic, and immunohistochemical features of 5 primary periorbital intraosseous cavernous vascular malformations.
  
  
  METHODS
  Retrospective interventional case series.
  
  
  METHODS
  Clinical and operative records and radiographic images were reviewed. Histopathologic slides were evaluated with hematoxylin-eosin, trichrome, and elastin stains. Immunohistochemical studies were performed with a spectrum of monoclonal antibodies directed at antigens of vascular cells.
  
  
  RESULTS
  Three men and 2 women ranged in age from 36 to 64 years. Vision was unaffected and there was no proptosis or globe displacement. The slow-growing lesions measured 13-25 mm in greatest diameter (mean 16.4 mm). Computed tomographic studies revealed that 2 lesions were situated in the maxillary bone, 2 in the frontal, and 1 in the zygoma, all anteriorly and with circumscribed, lucent, honeycombed, or sunburst characteristics. Histopathologically the lesions were composed of cavernous or telangiectatic channels; 1 showed advanced fibrotic vascular involution. Immunohistochemistry demonstrated CD31/34 positivity for vascular endothelium and D2-40 negativity for lymphatic endothelium. A typically thin mural myofibroblastic cuff was smooth muscle actin positive, weakly calponin positive, and desmin negative. Glucose transporter-1 and Ki-67 were negative in the endothelium.
  
  
  CONCLUSIONS
  Intraosseous vascular lesions resemble orbital cavernous venous malformations (not true hemangiomas), except that their vascular walls are thinner owing to the constraints imposed by neighboring bone spicules, which limit the amount of interstitium from which mural myofibroblasts can be recruited. The bony trabeculae conferred the honeycomb or sunburst appearances observed radiographically. En bloc excision of these lesions was successful and avoided complications (mean follow-up, 46 months).
authors.affiliation: David G. Cogan Laboratory of Ophthalmic Pathology, Boston, Massachusetts; Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; Harvard Medical School, Boston, Massachusetts.
authors.forename: Xiang Q
authors.initials: XQ
authors.lastname: Werdich
authors.affiliation: David G. Cogan Laboratory of Ophthalmic Pathology, Boston, Massachusetts; Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; Harvard Medical School, Boston, Massachusetts. Electronic address: fred_jakobiec@meei.harvard.edu.
authors.forename: Frederick A
authors.initials: FA
authors.lastname: Jakobiec
authors.affiliation: Department of Radiology, Boston, Massachusetts; Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; Harvard Medical School, Boston, Massachusetts.
authors.forename: Hugh D
authors.initials: HD
authors.lastname: Curtin
authors.affiliation: Ophthalmic Plastic Surgery, Boston, Massachusetts; Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; Harvard Medical School, Boston, Massachusetts.
authors.forename: Aaron
authors.initials: A
authors.lastname: Fay
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000378:metabolism; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D008875:Middle Aged; D009916:Orbital Diseases / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D012189:Retrospective Studies; D012886:Skull / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D013131:Spine / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology; D014057:Tomography, X-Ray Computed; D054079:Vascular Malformations / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology
chemical_list: D015415:Biomarkers