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Brian D. Alder, Jason A. Liss; Bilateral Orbital Epithelioid Hemangioma. Invest. Ophthalmol. Vis. Sci. 2011;52(14):719.
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Epithelioid hemangioma (EH) is a benign abnormal proliferation of endothelial cells usually arising in the head and neck region. While similar to Kimura disease, clinical and histologic features can be used to distinguish the two. EH rarely involves the orbit and has never before been reported to occur bilaterally. We present the first reported case of bilateral EH of the orbit.
The patient’s chart was reviewed and summarized in the following case report.
A 38 year old healthy female presented with subacute onset of left sided painless proptosis, chemosis and diplopia. Exam showed a well-appearing, afebrile female with visual acuity of 20/30 in the right eye and 20/60 in the left eye. Exam of the left orbit revealed a palpable, nontender, superolateral orbital mass with inferior globe displacement (figure 1), 4mm of relative left-sided proptosis, and near complete left external ophthalmoplegia. Optic nerves appeared normal in both eyes. CT scan showed a poorly defined, enhancing mass in the region of the lacrimal gland, measuring 3x2cm with no evidence of osseous destruction (figure 2). The patient underwent near-total excisional biopsy of the rapidly enlarging mass and histology showed an abnormal vascular and T-cell dominant lymphocyte proliferation consistent with EH. Several months later, the patient presented with complaints of fullness of the right eye. Eye exam was unimpressive, but repeat CT scan showed an enhancing, extraconal, superotemporal mass measuring 1.7x1.1cm in the right orbit, as well as partial regrowth of the left orbital mass. The patient underwent biopsy of the right orbital mass, and histology showed findings similar to that in the left orbit, consistent with EH. The patient received an oral steroid taper and is without clinical evidence of regrowth after 2 months. She will undergo serial CT scans to follow the size of the lesions.
This patient presented with orbital disease with a broad differential diagnosis including infection, inflammation and neoplasm. The diagnosis of EH, made by biopsy, was a surprising result. When the disease was found to be bilateral, suspicion of a misdiagnosed inflammatory condition was strongly raised, but careful review of the pathology confirmed the first reported case of bilateral EH. Kimura disease has been reported to occur in the orbits bilaterally, but the combination of specific histologic features and a lack of peripheral eosinophilia rule out Kimura disease. EH is generally treated with surgical excision, but given the infiltrative nature of these lesions, should they recur, consideration is being given to treatment with external beam radiation.
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