S1 Guideline Cutaneous Angiosarcomas – Update 2021

    Thomas Vogt, F. Max Müller, Patrick Melchior, Christian Rübe, Selma Ugurel, Tobias‐Torsten Schimming, Jochen Utikal, Stefan Eßer, Doris Helbig, Eva Hadaschik, Bernd Kasper, Stephan Grabbe
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    TLDR Cutaneous angiosarcomas require a combined treatment approach, but prognosis is generally poor.
    The 2021 update on guidelines for cutaneous angiosarcomas (AS) addressed the treatment and prognosis of this rare malignant tumor, which accounted for 1-2% of all soft tissue sarcomas. The document discussed the challenges in diagnosing AS due to non-specific symptoms and highlighted the importance of histopathological confirmation. It emphasized a multimodal treatment approach, combining surgery, radiotherapy, and chemotherapy, although the effectiveness of surgery was debated due to the tumor's multifocal nature. Adjuvant radiotherapy was crucial for improved local control, and chemotherapy, particularly with taxanes, showed potential benefits. Emerging therapies like trabectedin and pazopanib were promising but experimental. The prognosis for AS was generally poor, with a ten-year survival rate of 13.8%, though localized cases had better outcomes. The guideline also stressed the need for individualized follow-up and psychosocial care for patients.
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