Angiosarcoma of the head and neck

Angiosarcomas are uncommon tumors of the skin and soft tissues, but occur in endothelial cells of almost any internal organ. When they occur in the skin, the head and neck are the most common sites of origin. Nothing is pathognomonic about their clinical presentation, and the pathologic diagnosis can only be established by biopsy. The frequent multicentric nature of the tumor and propensity for extensive subcutaneous spread require wide treatment fields, whatever the modality of therapy, and account for the low survival reported. Four cases and their outcome are reported, with only one patient achieving an apparent cure following irradiation therapy. Based on experience and extensive review of the literature, a best therapeutic approach cannot be recommended. Since the outlook for cure is so unfavorable, there appears to be a need for cooperative studies using surgery, irradiation, and perhaps, chemotherapy.