Angiosarcomas (AS) are rare tumors of vascular origin with a variable behavior and overall poor prognosis in the metastatic setting. We sought to assess the outcomes of patients with metastatic AS treated with systemic chemotherapy in an attempt determine the ideal sequence of available standard agents.

We performed a retrospective analysis of patients with metastatic AS treated at Memorial Sloan Kettering Cancer Center between 1987 and 2012 and collected their correlative clinical information. Outcomes and efficacy measurements of first-line and subsequent lines of treatment were analyzed.

Among 119 pts with metastatic AS, the median age was 61 years and the female/male ratio was 1.4. The most frequent primary sites were chest wall/breast (n=37, 31%), viscera (n=26, 22%) and head/neck (n=24, 20%). There were 28(24%) patients with radiation -associated AS (RT-AS). The median OS was 12.1 months. We identified 73 (61%) and 46 (39%) patients that received ≥2 lines and ≥3 lines of therapy, respectively. The most commonly used agents included taxanes (T) and anthracyclines (A) with 62% of patients receiving either agent. Median times to tumor progression (TTP) were 3.5m for first line, 3.7m for 2nd line and 2.7m for 3^rd line. Overall response rate to 1st line was 30% and less than 10% in subsequent lines. No objective responses were documented in lines 5–7. Doxorubicin, liposomal doxorubicin and taxanes resulted in similar response rates and survival.

Despite reasonable response rates in the first line setting, benefit from systemic therapy is short-lived in metastatic AS and outcomes are poor. Doxorubicin, liposomal doxorubicin and taxanes are reasonable choices for first-line and any sequential use of these drugs in monotherapy is appropriate. Further exploration of the molecular pathophysiology to better identify better therapeutic strategies is essential.