Lymphangiosarcoma of the Scalp

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.