What are treatment recommendations for angiosarcoma?

Updated: Apr 29, 2019
  • Author: from Memorial Sloan-Kettering - Mrinal M Gounder, MD; Chief Editor: Edwin Choy, MD, PhD  more...
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Answer

Answer

Treatment is wide excision and irradiation; however, angiosarcoma is highly aggressive and has a propensity for local recurrence, multifocal spread, and early hematogenous dissemination.

Angiosarcomas have a 5-y overall survival rate of 10-30%; radiation-associated angiosarcomas are thought to have worse outcomes.

Systemic treatment of angiosarcomas includes doxorubicin or ifosfamide, with responses in the range of 10-20%; angiosarcomas are also highly sensitive to taxanes; pegylated-liposomal doxorubicin has also been shown to have activity in this disease. [33]

A phase II clinical trial of 30 patients with unresectable or metastatic angiosarcoma (ANGIOTAX study) demonstrated a 2-mo and 4-mo progression-free survival of 74% and 42%, respectively, with weekly paclitaxel administered at a dose of 80 mg/m2 on days 1, 8, and 15 in a 4-wk cycle; an overall response rate of 19% was observed. [34]

Docetaxel has also been shown to be promising, with 6 out of 9 patients treated achieving major responses. [35]

A phase II trial of sorafenib that included 37 patients with vascular sarcomas achieved one CR, four partial responses (PR), and an overall response rate of 14%; overall median progression-free survival was 3.2 mo; the median OS was 14.3 mo; patients with angiosarcoma had the greatest degree of tumor shrinkage overall of all vascular sarcoma subtypes. [36]

Single-agent bevacizumab can achieve a response rate of 12% and tumor stabilization in 62%. [37]


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