Abstract
Angiosarcoma is an uncommon malignancy, accounting for only 2% of all sarcomas. Malignant hemangioendothelioma (MHE) accounts for more than 80% of all cutaneous angiosarcoma with a more dismal prognosis. Many treatment options do exist, but no standard treatment method had been discovered thus far. Most of the cases thus far have been treated with wide local excision followed by radiation or interleukin-2 based immunotherapy without considering the pathology of the primary tumor. However, it is very important to consider pathological features, for IL2-based immunotherapy is effective only in the lesions which are so-called plaque lesions, with higher differentiation and presence of tumor infiltrating lymphocytes (TIL) . On the other hand, poorly differentiated tumors without TIL should be treated with other options. Even if an appropriate treatment will lead to a complete remission of the primary tumor, distant metastasis frequently occurs within 1-2 years and becomes lethal. Effective adjuvant therapy for angiosarcoma should be established to inhibit frequent distant metastasis, [Skin Cancer (Japan) 2006; 21: 279-285]
