10 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Angiosarcoma is an aggressive vascular tumour, a cancer of the cells that line blood and lymph vessels. It grows as bruise-like patches on the scalp of older people, in breasts treated years earlier with radiotherapy, or inside organs. Surgery and radiotherapy are used where possible, weekly paclitaxel is the most active drug, and immunotherapy helps a minority with the sun-damaged scalp form.
Angiosarcoma is a high-grade endothelial malignancy with three main clinical settings: cutaneous angiosarcoma of the scalp and face in elderly patients, carrying an ultraviolet mutational signature and high tumour mutational burden; secondary angiosarcoma after breast radiotherapy or in chronic lymphoedema, typically driven by MYC amplification; and primary visceral angiosarcoma of the liver, heart, spleen and breast. Tumours are multifocal and infiltrative, margins are hard to secure, and local recurrence and lung metastasis are common even after apparently complete treatment.
Localised disease is treated with wide surgery and radiotherapy, often combined because neither alone controls the diffuse scalp form. For advanced disease, weekly paclitaxel established itself through the French ANGIOTAX phase 2 trial and is used first line or as neoadjuvant treatment for scalp disease; doxorubicin and liposomal doxorubicin are alternatives, and gemcitabine and pazopanib are used later. Adding bevacizumab to paclitaxel (ANGIOTAX-PLUS) and the endoglin antibody TRC105 to pazopanib (TAPPAS) did not improve outcomes.
| Setting | Approach | Guideline |
|---|---|---|
| Localised cutaneous (scalp, face) | Wide excision where feasible plus wide-field radiotherapy; neoadjuvant or definitive weekly paclitaxel with radiotherapy when surgery is not possible. | not mapped |
| Radiation-associated breast angiosarcoma | Total mastectomy with wide skin excision; consider neoadjuvant paclitaxel; re-irradiation is limited by prior dose. | not mapped |
| Advanced, first line | Weekly paclitaxel (ANGIOTAX) or doxorubicin-based chemotherapy; liposomal doxorubicin in frail patients. | not mapped |
| Later lines | Gemcitabine, pazopanib; checkpoint inhibitors (nivolumab plus ipilimumab) for cutaneous scalp and face disease, off label or in trials. | not mapped |