Abstract
SummaryAngiosarcomas are rare aggressive endothelial tumours, and are associated with a poor prognosis. Due to their vascular nature, there is great interest in their response to anti-angiogenic agents. A number of small prospective studies have reported angiosarcoma response to vascular-targeted agents, including agents that target vascular endothelial growth factor. To date, the response to these agents has been disappointing, and similar to the response observed in other soft tissue sarcoma subtypes. This short review will summarise the recent data in this field.
Highlights
As angiosarcomas are endothelial tumours, there is interest in the response of angiosarcoma to vascular-targeted agents. This short review will summarise the recent data in this field
Similar to soft tissue sarcomas (STS) response to pazopanib, an unplanned pooled analysis of non-adipocytic STS cohorts reported a median progression-free survival (PFS) 4.0 months with regorafenib compared to 1.0 months with placebo (HR 0.36, 95% CI 0.25–0.53; p < 0.0001)
Non-adipocytic STS response to TRC105 in combination with pazopanib was studied in a phase I/II study [17]; response to treatment was observed in 8/9 angiosarcoma patients, including two durable CR
Summary
Angiosarcoma response to bevacizumab monotherapy (15 mg/kg 3 weekly) was studied in a single arm phase II study, which recruited patients with epithelioid haemangioendothelioma [5]. 23 patients with advanced angiosarcoma were recruited, and in these patients, the response rate was 9% (2/23); 43% (10/23) of patients progressed within 6 weeks, and the median PFS was 3 months. The addition of bevacizumab (10 mg/kg 2 weekly) to weekly paclitaxel (90 mg/m2) was studied in a noncomparative, open-label, randomised phase II study [6]. The addition of bevacizumab to paclitaxel was associated with higher rates of serious toxicity (44% vs 22%), and more patients receiving combination treatment required dose reductions (56% vs 35%). The addition of bevacizumab to weekly paclitaxel is not recommended
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