Abstract
The classification of certain vascular bone tumors that show an epithelioid cytologic appearance remains confusing, with overlap in features of epithelioid hemangioma, hemangioendothelioma and epithelioid hemangioendothelioma. Radiographs of a 27-year-old woman who presented with ankle pain showed an expanded lytic-sclerotic lesion in the distal left fibula. Magnetic resonance imaging showed an intramedullary lesion with a small lateral intracortical component. The lesion was hypo- to isointense to muscle on T1-weighted images and heterogeneously hyperintense on T2-weighted images. Initial incisional biopsy was inconclusive. Open biopsy showed hemangioendothelioma with epithelioid morphology, and the lesion was completely resected with reconstruction using a peroneal fibular rotation graft. Examination of the resected specimen showed focal hemangioendothelioma with an epithelioid phenotype arising in a hemangioma. This case illustrates the difficulty and pitfalls of making the correct diagnosis on the basis of a small biopsy specimen.
Similar content being viewed by others
References
Dorfman HD, Steiner GC, Jaffe HC. Vascular tumors of bone. Hum Pathol 1975; 3:349–376.
Wenger DE, Wold LE. Malignant vascular lesions of bone: radiologic and pathologic features. Skeletal Radiol 2000; 29:619–631.
Bhagavan BS, Dorfman HD, Murthy MS, Eggleston JC. Intravascular bronchiolo-alveolar tumor (IVBAT): a low grade sclerosing epithelioid angiosarcoma of lung. Am J Surg Pathol 1982; 6:41–52.
Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM. Epithelioid hemangioendothelioma and related lesions. Semin Diagn Pathol 1986; 3:259–287.
Tsang WY, Chan JK. The family of epithelioid vascular tumors. Histol Histopathol 1993; 8:187–212.
Ignacio EA, Palmer KM, Mathur SC, Schwartz AM, Olan WJ. Epithelioid hemangioendothelioma of the lower extremity. Radiographics 1999; 19:531–537.
Evans HL, Raymond AK, Ayala AG. Vascular tumors of bone: a study of 17 cases other than ordinary hemangioma, with an evaluation of the relationship of hemangioendothelioma of bone to epithelioid hemangioma, epithelioid hemangioendothelioma, and high-grade angiosarcoma. Hum Pathol 2003; 34:680–689.
O’Connell JX, Kattapuram SV, Mankin HJ, Bhan AK, Rosenberg AE. Epithelioid hemangioma of bone: a tumor often mistaken for low-grade angiosarcoma or malignant hemangioendothelioma. Am J Surg Pathol 1993; 17:610–617.
De Young BR, Wick MR, Fitzgibbon JF, Sirgi KE, Swanson PE. CD31: an immunospecific marker for endothelial differentiation in human neoplasms. Appl Immunohistochem 1993; 1:97–100.
Kleer CG, Unni KK, McLeod RA. Epithelioid hemangioendothelioma of bone. Am J Surg Pathol 1996; 20:1301–1311.
Author information
Authors and Affiliations
Corresponding author
Additional information
Presented (case no. 17) at the 26th Closed Meeting of the International Skeletal Society, Seattle, Washington, USA, August 1999
Rights and permissions
About this article
Cite this article
Shah, Z.K., Peh, W.C.G., Shek, T.W.H. et al. Hemangioendothelioma with an epithelioid phenotype arising in hemangioma of the fibula. Skeletal Radiol 34, 750–754 (2005). https://doi.org/10.1007/s00256-005-0912-0
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00256-005-0912-0