Background: Lymphangiosarcoma is a rare, aggressive malignancy that originates from the endothelial cells lining lymphatic vessels and carries an extremely poor prognosis. Its clinical and histologic features are often indistinguishable from angiosarcoma.

Objective: We sought to better characterize the clinical and histologic features of lymphangiosarcoma.

Methods: Case report and review of the literature.

Results: A number of immunohistochemical markers, including Von Willebrand factor, Ulex europaeus agglutinin 1, CD31, VEGFR-3, D2-40, Prox-1, can be used to help differentiate lymphatic from vascular tissue.

Conclusions: Recent characterization of several new biologic markers has allowed greater differentiation between these tumors and may provide new therapeutic targets for treatment.

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http://dx.doi.org/10.2310/7750.2013.13025DOI Listing

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