What is the efficacy of chemotherapy for the treatment of soft-tissue angiosarcoma (AS)?

Updated: Sep 04, 2018
  • Author: Maria Belén Carsi, MD, PhD, FRCS; Chief Editor: Edwin Choy, MD, PhD  more...
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Ravi et al report exceptional response to treatment with pazopanib in a patient with angiosarcoma that harbored amplification of vascular endothelial growth factor receptor (VEGFR) and that had not responded to sorafenib. These authors suggest that a subset of patients with angiosarcoma with genomic alterations in vascular signaling genes may respond well to pazopanib. [22]

Multiple randomized studies using doxorubicin-based chemotherapy fail to show a survival benefit from neoadjuvant chemotherapy. However, a meta-analysis suggests improved local control and disease-free survival with chemotherapy, but no survival advantage. [12, 23]

Response to preoperative chemotherapy is only 40-50%, with the most active regimens, and toxicity is significant. Consequently, specialists reserve preoperative chemotherapy for patients with high-grade lesions. The regimen is continued in those patients who respond with tumor shrinkage after two to three courses of multiagent chemotherapy after tumor resection.

Pasquier et al reported effective treatment in seven patients with advanced angiosarcoma using the combination of twice-daily propranolol (40 mg) and weekly metronomic vinblastine (6 mg/m2) and methotrexate (35 mg/m2). All patients responded; one patient showed a complete response and three showed very good partial responses. Median progression-free and overall survival was 11 months (range 5–24) and 16 months (range 10–30), respectively. [24] In January 2017, propranolol was granted orphan drug status in Europe for the treatment of soft tissue sarcoma. [25]

Similarly, Amaya et al reported increased progression-free and overall survival in patients with metastatic angiosarcoma who received treatment with nonselective beta-blockers. Eight of their patients were treated with propranolol (20 to 100 mg/day) and one patient was treated with carvedilol (6.25 mg/day). [26]

Offer patients with unresponsive tumors different treatment regimens. Response to neoadjuvant chemotherapy can be observed, but it does not always correlate with radiographic response.

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