Cutaneous angiosarcoma.

Abstract

OBJECTIVE: To discuss the treatment and outcomes for cutaneous angiosarcoma. METHODS: Review of the pertinent literature. RESULTS: Cutaneous angiosarcoma is a rare, aggressive malignancy with a poor prognosis. It usually arises in the scalp or face and is locally advanced at presentation. Patients are most often white, male, and elderly. A subset of patients presents with multifocal disease and/or positive regional nodes. Although the optimal treatment is surgery followed by wide-field radiotherapy (RT), the disease is frequently so extensive at diagnosis that it is not completely resectable. Even after optimal local-regional treatment, there is a relatively high likelihood of a local recurrence at the margins of the RT fields. The probability of hematogenous dissemination is relatively high. Limited data suggest that chemotherapy may be useful for palliation with progression-free survival rates ranging from 1 to 5 months. The 5-year local-regional control rates are approximately 40% to 50%, the 5-year distant metastasis-free survival rates range from 20% to 40%, and the 5-year survival rates range from 10% to 30%. CONCLUSION: Cutaneous angiosarcoma is a rare, aggressive malignancy that is optimally treated with resection and wide-field postoperative RT. The likelihood of local-regional failure is high, as is the risk of distant relapse. Chemotherapy may be useful for short-term palliation.