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C.V. Duss, N. Mirani, P.D. Langer; Intraosseous Hemangioma of the Orbit: Clinical Presentation, Pathologic Diagnosis, and Surgical Management of a Rare Orbital Tumor . Invest. Ophthalmol. Vis. Sci. 2006;47(13):4704.
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To review the presentation, diagnosis, and management of two cases of orbital intraosseous hemangiomas.
Charts of two patients diagnosed with orbital intraosseous hemangiomas were reviewed.
Case 1: A 28 year old woman presented with a longstanding left inferior orbital rim mass which had recently increased in size. Examination revealed a firm, nodular, palpable (1.5x1cm), nonmobile mass of the left anterior inferior orbit; a CT scan demonstrated a partially ossified, destructive bony lesion of the inferior orbital rim. Excisional biopsy was performed, and a hard, rounded protuberance of the inferior orbital rim was excised. Pathologic examination revealed endothelial lined sinuses and blood filled channels within the substance of the bone of the orbital rim consistent with a benign intraosseous capillary hemangioma. Case 2: An 82 year old woman presented with a mass over the left lateral orbital rim which was increasing in size. She denied pain, vision changes or diplopia. On examination, a fixed, firm, rubbery mass on the left lateral orbital rim was noted, as well as several cherry hemangiomas on her face. A CT scan demonstrated a soft tissue density mass at the left orbital rim with erosion of the adjacent frontal process of the zygoma. MR images revealed a high signal intensity lesion in the frontal process of the left zygoma. Additionally, similar lesions were visualized in the left parietal bone and the left frontal lobe. Excisional biopsy of a purplish encapsulated mass encrypted in the bony orbital rim was performed. Pathologic examination demonstrated endothelial cell lined blood vessels within bony trabeculae consistent with an intraosseous hemangioma.
Intraosseous hemangiomas are rare tumors most commonly found in the skull which often excavate bone. They are thought to be hamartomas of vascular channels derived from undifferentiated mesenchymal tissue and are most frequently reported in adults in the fifth to sixth decade with a 3:1 female:male ratio. Only 30 cases have been reported to occur in the orbit. Most orbital lesions involve the frontal or zygomatic bones, and in 12–15% of cases there are multiple lesions. They are generally painless and without symptoms unless mass effect plays a role. The chief concern in the differential diagnosis is a new bone metastasis; treatment may be conservative once the diagnosis is established.
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