{"entity":{"id":"angiosarcoma","kind":"cancer","name":"Angiosarcoma","aka":["Haemangiosarcoma","Lymphangiosarcoma","Stewart-Treves syndrome (lymphoedema-associated angiosarcoma)","Radiation-associated angiosarcoma"],"tldr":"Angiosarcoma is an aggressive 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.","summary":"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.\n\nLocalised 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.\n\nCheckpoint inhibitors produce durable responses in a subset of cutaneous scalp and face angiosarcomas, consistent with their ultraviolet-driven mutational load, in the DART ipilimumab-nivolumab basket and in case series, but the randomised Alliance A091902 trial did not show that adding nivolumab to paclitaxel improved progression-free survival across all angiosarcomas. Propranolol, targeted anti-angiogenic combinations and the international Angiosarcoma Project patient-partnered genomics effort are the main lines of research.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Angiosarcoma","links":[{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Angiosarcoma"}],"tags":["subtype-page"],"related":["vascular-tumours","kaposi-sarcoma","epithelioid-haemangioendothelioma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"sarcoma","burden":"About one to two percent of soft tissue sarcomas; arises in the sun-damaged scalp and face of older people, in the irradiated breast, in chronically swollen limbs and in the liver, heart and spleen. It spreads early and median survival with metastatic disease is under a year in most series.","subtypes":["Cutaneous angiosarcoma of the scalp and face (ultraviolet signature, high mutational burden)","Radiation-associated angiosarcoma of the breast (MYC-amplified)","Lymphoedema-associated angiosarcoma (Stewart-Treves)","Primary breast angiosarcoma (younger women)","Visceral angiosarcoma (liver, heart, spleen, bone)"],"biomarkers":["MYC amplification (secondary angiosarcoma)","Tumour mutational burden and ultraviolet signature (scalp and face; immunotherapy response)","CD31, ERG and FLI1 endothelial markers","KDR and PLCG1 mutations","PTPRB and PLCG1 in secondary breast tumours"],"standardOfCare":[{"setting":"Localised cutaneous (scalp, face)","approach":"Wide excision where feasible plus wide-field radiotherapy; neoadjuvant or definitive weekly paclitaxel with radiotherapy when surgery is not possible.","refs":["imrt-igrt","paclitaxel"]},{"setting":"Radiation-associated breast angiosarcoma","approach":"Total mastectomy with wide skin excision; consider neoadjuvant paclitaxel; re-irradiation is limited by prior dose.","refs":["paclitaxel","doxorubicin"]},{"setting":"Advanced, first line","approach":"Weekly paclitaxel (ANGIOTAX) or doxorubicin-based chemotherapy; liposomal doxorubicin in frail patients.","refs":["paclitaxel","angiotax","doxorubicin","pegylated-liposomal-doxorubicin"]},{"setting":"Later lines","approach":"Gemcitabine, pazopanib; checkpoint inhibitors (nivolumab plus ipilimumab) for cutaneous scalp and face disease, off label or in trials.","refs":["gemcitabine","pazopanib","nivolumab","ipilimumab","checkpoint-inhibitor"]}],"stateOfArt":["Weekly paclitaxel is the reference systemic therapy, with responses concentrated in cutaneous scalp and face disease.","Sun-damaged scalp angiosarcoma responds to checkpoint blockade, making it one of the few sarcomas where immunotherapy works.","The patient-partnered Angiosarcoma Project has sequenced hundreds of tumours from home-shipped samples, defining the MYC-amplified and ultraviolet-driven groups."],"history":[{"year":1948,"title":"Stewart and Treves describe angiosarcoma in the lymphoedematous arm after mastectomy","refs":[]},{"year":2008,"title":"ANGIOTAX: weekly paclitaxel established as active in angiosarcoma","refs":["angiotax","paclitaxel"]},{"year":2010,"title":"MYC amplification found in radiation-associated and lymphoedema angiosarcoma","refs":[]},{"year":2015,"title":"ANGIOTAX-PLUS: adding bevacizumab to paclitaxel does not improve outcomes","refs":["paclitaxel","bevacizumab"]},{"year":2020,"title":"Angiosarcoma Project reports ultraviolet signature and immunotherapy responses in scalp and face tumours","refs":["checkpoint-inhibitor"]},{"year":2022,"title":"TAPPAS: TRC105 plus pazopanib fails to beat pazopanib","refs":["pazopanib"]}],"pipeline":["checkpoint-inhibitor","nivolumab","ipilimumab","pazopanib"],"openProblems":["Multifocal scalp disease escapes even wide surgery and radiotherapy.","Median survival with metastases remains under a year.","Immunotherapy benefit is limited to the ultraviolet-driven subset and has not been confirmed in a randomised trial."],"parent":"vascular-tumours"},"route":"/cancers/angiosarcoma/","neighbours":{"cancer":[{"id":"epithelioid-haemangioendothelioma","kind":"cancer","name":"Epithelioid haemangioendothelioma","route":"/cancers/epithelioid-haemangioendothelioma/"},{"id":"kaposi-sarcoma","kind":"cancer","name":"Kaposi sarcoma","route":"/cancers/kaposi-sarcoma/"},{"id":"vascular-tumours","kind":"cancer","name":"Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma)","route":"/cancers/vascular-tumours/"}],"trial":[{"id":"angiotax","kind":"trial","name":"ANGIOTAX","route":"/trials/angiotax/"}],"drug":[{"id":"bevacizumab","kind":"drug","name":"Bevacizumab","route":"/drugs/bevacizumab/"},{"id":"doxorubicin","kind":"drug","name":"Doxorubicin","route":"/drugs/doxorubicin/"},{"id":"gemcitabine","kind":"drug","name":"Gemcitabine","route":"/drugs/gemcitabine/"},{"id":"ipilimumab","kind":"drug","name":"Ipilimumab","route":"/drugs/ipilimumab/"},{"id":"nivolumab","kind":"drug","name":"Nivolumab","route":"/drugs/nivolumab/"},{"id":"paclitaxel","kind":"drug","name":"Paclitaxel / nab-paclitaxel","route":"/drugs/paclitaxel/"},{"id":"pazopanib","kind":"drug","name":"Pazopanib","route":"/drugs/pazopanib/"},{"id":"pegylated-liposomal-doxorubicin","kind":"drug","name":"Pegylated liposomal doxorubicin","route":"/drugs/pegylated-liposomal-doxorubicin/"}],"technology":[{"id":"checkpoint-inhibitor","kind":"technology","name":"Immune checkpoint inhibitors","route":"/technologies/checkpoint-inhibitor/"},{"id":"imrt-igrt","kind":"technology","name":"IMRT / IGRT (modern external beam)","route":"/technologies/imrt-igrt/"}]}}