Atypical hemangioendothelioma of venous origin. A clinicopathologic, angiographic, immunohistochemical, and ultrastructural study of two endothelial tumors within the concept of histiocytoid hemangioma.

Two patients, a 62-year-old man and a 50-year-old woman, both with deep-seated atypical endothelial tumors within the wide concept of histiocytoid hemangioma, are reported. In case 1, the tumor involved the brachial vein, and, in case 2, a medium-sized vein of the anterior neck. In both cases the involved vein was occluded. Angiography in case 1 suggested a tumor that was enclosed by the same fibrous sheath, the conjunctiva vasorum, that enclosed the occluded vein and its concomitant artery. Both tumors were solid, without conspicuous vascular differentiation by light microscopy. Such differentiation, however, was evident from the electron-microscopic examination, which showed tumor cells with endothelial features forming primitive vascular structures. Positive lectin histochemistry (Ulex Europeus I) and positive immunohistochemistry for factor-VIII-related antigen, actin, and vimentin also gave strong support for the endothelial differentiation of the tumor cells. Immunohistochemical studies of markers for histiocytic (alpha 1-antitrypsin, ferritin, lysozyme), epithelial (cytokeratin, epithelial membrane antigen), and neuroectodermal (S-100 protein) and skeletal muscle (myoglobin) differentiation were negative. At follow-up, after 7 years and 2 years, respectively, there were no signs of local recurrence or metastasis.