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January 1996

Angiosarcoma Metastatic to the Orbit

Author Affiliations

From the Department of Ophthalmology, W. K. Kellogg Eye Center (Drs Burnstine, Frueh, and Elner), and the Department of Pathology (Dr Elner), The University of Michigan, Ann Arbor.

Arch Ophthalmol. 1996;114(1):93-96. doi:10.1001/archopht.1996.01100130089017

Angiosarcoma is a rare malignant endothelial cell tumor. Few reports of primary orbital angiosarcoma exist in the literature. A 46-year-old woman had a 3-month history of progressive diplopia and right-sided exophthalmos. Magnetic resonance imaging showed a right intraconal mass, consistent with a cavernous hemangioma. Ultrasound examination showed an isolated mass with moderate vascularity, consistent with a metastatic lesion. Sub-total excision through a lateral orbitotomy was performed. Tissue was stained with hematoxylineosin, periodic acid-Schiff, and immunohistochemical stains for factor VIII-related antigen and Ulex europaeus agglutinin I lectin. Lateral orbitotomy biopsy specimens showed an angiosarcoma, similar to a primary breast angiosarcoma that had been resected 2 years previously. The patient initially underwent orbital irradiation, which failed to control tumor progression. Subsequently, she underwent orbital exenteration for her only known metastasis. Despite a multidisciplinary and aggressive approach to this tumor, the tumor recurred, and the patient died.

Bardwil JM, Mocega EE, Butler JJ, Russin DJ.  Angiosarcomas of the head and neck region . Am J Surg . 1968;116:548-553.Article
Enzinger FM, Weiss SW. Malignant Vascular Tumors: Soft Tissue Tumors . St Louis, Mo: CV Mosby Co; 1988:548-580.
Messmer EP, Font RL, McCrary JA, Murphy D.  Epithelioid angiosarcoma of the orbit presenting as Tolosa-Hunt syndrome: a clinicopathologic case report with review of the literature . Ophthalmology . 1983;90:1414-1421.Article
Hufnagel T, Ma L, Kuo T.  Orbital angiosarcoma with subconjunctival presentation: report of a case and literature review . Ophthalmology . 1987;94:72-77.Article
Shuangshoti S, Chayapum P, Suwanwela N, Suwanwela C.  Unilateral proptosis as a clinical presentation in primary angiosarcoma of the skull . Br J Ophthalmol . 1988;72:713-719.Article
Lyon DB, Tang TT, Kidder TM.  Epithelioid hemangioendothelioma of the orbital bones . Ophthalmology . 1992;99:1773-1778.Article
Bankaci M, Myers EN, Barnes L, DuBois P.  Angiosarcoma of the maxillary sinus: literature review and case report . Head Neck . 1979;1:274-280.Article
Wilson-Jones E.  Malignant angioendothelioma of the skin . Br J Dermatol . 1964;76:21-39.Article
Reed RJ, Palomeque FE, Hairston MA, Krementz ET.  Lymphangiosarcomas of the scalp . Arch Dermatol . 1966;94:396-402.Article
Steingaszner LC, Enzinger FH, Taylor HB.  Hemangiosarcoma of the breast . Cancer . 1965;18:352-361.Article
Maddox JC, Evans HL.  Angiosarcoma of skin and soft tissue: a study of forty-four cases . Cancer . 1981;48:1907-1921.Article
Benda JA, Al-Jurf AS, Benson AB.  Angiosarcoma of the breast following segmental mastectomy complicated by lymphedema . Am J Clin Pathol . 1986;87:651-655.
Silverman LR, Deligdisch L, Mandeli J, Greenspan EM.  Chemotherapy for angiosarcoma of the breast: case report of 30-year survival and analysis of the literature . Cancer Invest . 1994;12:145-155.Article
Henderson JW, Farrow GM.  Primary orbital hemangiopericytoma . Arch Ophthalmol . 1978;96:666-673.Article
Burgdorf WH, Mukai K, Rosai J.  Immunohistochemical identification of factor VIII-related antigen in endothelial cells of cutaneous lesions of alleged vascular nature . Am J Clin Pathol . 1981;75:167-171.
Walker RA.  Ulex europaeus I—peroxidase as a marker of vascular endothelium: its application in routine histopathology . J Pathol . 1985;146:123-127.Article
Nadji M, Gonzalez MS, Castro A, Morales AR.  Factor VIII-related antigen: an endothelial cell marker . Lab Invest . 1980;42:139.
Miettinen M, Holthofer H, Lehto V, Miettinen A.  Virtanen I. Ulex europaeus I lectin as a marker for tumors derived from endothelial cells . Am J Clin Pathol . 1983;79:32-36.
Ordonez NG, Batsakis JG.  Comparison of Ulex europaeus I lectin and factor VIII-related antigen in vascular lesions . Arch Pathol Lab Med . 1984;108:129-132.
Rosen PP, Kimmel M, Ernsberger D.  Mammary angiosarcoma: the prognostic significance of tumor differentiation . Cancer . 1988;62:2145-2151.Article
Donnell RM, Rosen PP, Lieberman PH, et al.  Angiosarcoma and other vascular tumors of the breast . Am J Surg Pathol . 1981;5:629-642.Article
Merino MJ, Berman M, Carter D.  Angiosarcoma of the breast . Am J Surg Pathol . 1983;7:53-60.Article