Angiosarcoma: A frequently recurrent and highly metastatic malignancy

Angiosarcoma is an uncommon, understudied soft tissue sarcoma of endothelial origin that accounts for only 1-2% of soft tissue sarcomas. These tumors represent a rare heterogeneous group of vascular neoplasms associated with an aggressive clinical behavior. Angiosarcomas occur in several clinical settings and are frequently multifocal with insidious growth patterns. These features make local control challenging and contribute to the generally poor prognosis of patients with these tumors. Up to 70% of angiosarcoma patients develop local recurrences and 36% of completely resected angiosarcoma patients develop distant failure. Additionally, 15% of patients present with metastatic disease at the time of diagnosis. Wide local surgical resection is the cornerstone of angiosarcoma treatment. Angiosarcomas have a high rate of systemic failure despite combined treatments such as surgery with radiation and/or systemic chemotherapy representing a major clinical challenge. Effective therapeutic strategies for these tumors are needed. Recent phase II trials suggest that paclitaxel may be a promising agent for the treatment of advanced angiosarcoma. Preclinical studies support a key role for angiogenic factors in angiosarcomas. Further investigations to determine the effectiveness of anti-angiogenic agents on the treatment of angiosarcomas is warranted.