Angiosarcoma: Treatment

Angiosarcoma: overview

This algorithm outlines treatment of angiosarcoma, whether cutaneous or visceral, divided by disease extent and line of therapy. Localized disease is treated with curative intent using wide resection with or without radiotherapy as part of multimodality care.

For advanced disease in the first line, two options are shown: weekly paclitaxel, especially for cutaneous and scalp primaries, and doxorubicin, linked to EORTC 62012. That trial found that adding ifosfamide to doxorubicin improved response and progression-free survival without an overall survival benefit in advanced soft-tissue sarcoma, supporting doxorubicin monotherapy as a standard first-line option. In later lines, the chart lists pazopanib, based on PALETTE, which improved progression-free survival versus placebo in pretreated soft-tissue sarcoma, and gemcitabine-based therapy or a checkpoint inhibitor, the latter noted for UV-driven tumors with high tumor mutational burden. The linked trials enrolled broad soft-tissue sarcoma populations rather than angiosarcoma specifically.

Trials behind this algorithm

• EORTC 62012 Judson I et al, Lancet Oncol, 2014; PMID: 24618336
• PALETTE Van der Graaf WT et al, Lancet, 2012; PMID: 22595799

Background reading: Sarcomas board review notes.