Angiosarcoma
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.
Overview
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.
Checkpoint 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.
State of the art
- 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.
Red cards
From the labels and guidelines behind the standard of care. Your team's thresholds win.- Emergency services nowBleeding or bruising
Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
- Emergency services nowHypophysitis or adrenal crisis
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
- Emergency services nowBowel perforation
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
- Emergency services nowFainting or palpitations
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
- Check before combiningFood and drink: Doxorubicin
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
- Check before combiningFood and drink: Pazopanib
Take on an empty stomach (1 hour before or 2 hours after food).
See all on the product pages:DoxorubicinGemcitabineIpilimumabNivolumabPaclitaxel / nab-paclitaxelPazopanibPegylated liposomal doxorubicin·Printable cards in the navigator
Anatomy and lymph node drainage
- Metaphysis, near the growth plate (osteosarcoma)
- Shaft (Ewing sarcoma)
- Deep soft tissue compartment
- Skeletal muscle (rhabdomyosarcoma)
- Neurovascular bundle (limb salvage decision)
Bone sarcomas favour the fast-growing ends of long bones (osteosarcoma) or the shaft (Ewing), soft tissue sarcomas the deep muscle compartments; spread is through the blood to the lungs, rarely via lymph nodes.
- Metaphysis, near the growth plate (osteosarcoma)
- Shaft (Ewing sarcoma)
- Deep soft tissue compartmentCutaneous 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)
- Skeletal muscle (rhabdomyosarcoma)
- Neurovascular bundle (limb salvage decision)
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Leiomyosarcoma, Liposarcoma, Synovial sarcoma, Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma, Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), Chondrosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Myxofibrosarcoma, Alveolar soft part sarcoma, Perivascular epithelioid cell tumour (PEComa), Epithelioid haemangioendothelioma, Malignant peripheral nerve sheath tumour (MPNST), Retroperitoneal sarcoma, Soft tissue sarcoma of the extremity (localised and advanced)
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.
Show survival figures (1)
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
- It spreads early and median survival with metastatic disease is under a year in most series.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Wide excision where feasible plus wide-field radiotherapy; neoadjuvant or definitive weekly paclitaxel with radiotherapy when surgery is not possible.
Total mastectomy with wide skin excision; consider neoadjuvant paclitaxel; re-irradiation is limited by prior dose.
Weekly paclitaxel (ANGIOTAX) or doxorubicin-based chemotherapy; liposomal doxorubicin in frail patients.
Gemcitabine, pazopanib; checkpoint inhibitors (nivolumab plus ipilimumab) for cutaneous scalp and face disease, off label or in trials.
Subtypes & biomarkers
top- 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)
- 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
How often this target appears
- 1948Stewart and Treves describe angiosarcoma in the lymphoedematous arm after mastectomy
- 2008ANGIOTAX: weekly paclitaxel established as active in angiosarcoma
- 2010MYC amplification found in radiation-associated and lymphoedema angiosarcoma
- 2015ANGIOTAX-PLUS: adding bevacizumab to paclitaxel does not improve outcomes
- 2020Angiosarcoma Project reports ultraviolet signature and immunotherapy responses in scalp and face tumours
- 2022TAPPAS: TRC105 plus pazopanib fails to beat pazopanib
Dated changes read from the records linked to this cancer: approvals, regulatory steps, reported trials, guideline versions and milestones. Newest first; no date is inferred.
All 8 changes by month →- 2026-09-17This recordAngiosarcomaFacts on this page last checked
When this page itself was last checked or edited.
- 2022MilestonePazopanibTAPPAS: TRC105 plus pazopanib fails to beat pazopanib
A milestone in how this cancer is treated.
- 2020MilestoneImmune checkpoint inhibitorsAngiosarcoma Project reports ultraviolet signature and immunotherapy responses in scalp and face tumours
A milestone in how this cancer is treated.
- 2015MilestonePaclitaxel / nab-paclitaxelANGIOTAX-PLUS: adding bevacizumab to paclitaxel does not improve outcomes
A milestone in how this cancer is treated.
- 2010MilestoneAngiosarcomaMYC amplification found in radiation-associated and lymphoedema angiosarcoma
A milestone in how this cancer is treated.
- 2008Trial resultANGIOTAXANGIOTAX reported
Primary endpoint met: most patients progression-free at two months; responses mainly in cutaneous scalp and face disease.
What is in development for Angiosarcoma, drawn from the whole corpus: 2 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Technologies being tested · 1
Trials reported · 1
- ANGIOTAX · phase 2 · 2008 · completed
Open problems and what is being done
Multifocal scalp disease escapes even wide surgery and radiotherapy.
Median survival with metastases remains under a year.
and how the field plans to fix it →What is being done about thisAdvanced and metastatic diseaseAvailable now- IMRT / IGRT (modern external beam)Standard of care
In trialsNothing recorded yet.
Ideas and roadmapsNothing recorded yet.
Also on OnCo: Atlas of advanced disease · Invasion and metastasis.
Immunotherapy benefit is limited to the ultraviolet-driven subset and has not been confirmed in a randomised trial.
Trials
topTrials recruiting now
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Landmark trials
Expert centres
topExpert centres
Amsterdam · cancer center | Netherlands | none recorded | 0 | 1,451 | 25,873 | #45 | |
Wuhan · hospital | China | none recorded | 0 | 2,478 | 31,527 | - | |
Utrecht · cancer center | Netherlands | none recorded | 0 | 1,422 | 20,323 | - | |
Beijing · cancer center | China | none recorded | 0 | 1,344 | 18,195 | - | |
Leiden · university | Netherlands | none recorded | 0 | 1,245 | 16,125 | - | |
Tianjin · cancer center | China | none recorded | 0 | 1,219 | 11,455 | - | |
Hangzhou · cancer center | China | none recorded | 0 | 1,219 | 17,635 | - | |
Lyon · cancer center | France | none recorded | 0 | 1,071 | 14,301 | - | |
Rozzano (Milan) · hospital | Italy | none recorded | 0 | 1,031 | 10,720 | - | |
Zhengzhou · cancer center | China | none recorded | 0 | 970 | 12,409 | - | |
Padua · cancer center | Italy | none recorded | 0 | 962 | 12,326 | - | |
Naples · cancer center | Italy | none recorded | 0 | 961 | 15,028 | - | |
Changsha · cancer center | China | none recorded | 0 | 930 | 14,477 | - | |
Jinan · cancer center | China | none recorded | 0 | 920 | 7,804 | - | |
New Haven, CT · cancer center | United States | 0 | 852 | 16,798 | - |
These are the things we can measure; they are not a ranking of quality. Each column is a field on the institution record or a count over what OnCo has linked; a centre that treats many patients with Angiosarcoma but is thinly recorded here will look small.
Not known: OnCo holds no case-volume or outcome figures for centres, so none are shown. Where a national audit or registry publishes them, the centre's page links to it. Default order: Newsweek rank, then trials for this cancer, then research output.
Questions to ask
topQuestions to ask your oncologist about Angiosarcoma
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example MYC amplification, Tumour mutational burden and ultraviolet signature, CD31, ERG and FLI1 endothelial markers, KDR and PLCG1 mutations, PTPRB and PLCG1 in secondary breast tumours), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Cutaneous angiosarcoma of the scalp and face, Radiation-associated angiosarcoma of the breast, Lymphoedema-associated angiosarcoma.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Localised cutaneous (scalp, face)
- For my situation (localised cutaneous (scalp, face)), which of the standard options do you recommend and why?Why: Guideline options include: Wide excision where feasible plus wide-field radiotherapy; neoadjuvant or definitive weekly paclitaxel with radiotherapy when surgery is not possible.
- Am I a candidate for Paclitaxel / nab-paclitaxel, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Radiation-associated breast angiosarcoma
- For my situation (radiation-associated breast angiosarcoma), which of the standard options do you recommend and why?Why: Guideline options include: Total mastectomy with wide skin excision; consider neoadjuvant paclitaxel; re-irradiation is limited by prior dose.
- Am I a candidate for Paclitaxel / nab-paclitaxel, Doxorubicin, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced, first line
- For my situation (advanced, first line), which of the standard options do you recommend and why?Why: Guideline options include: Weekly paclitaxel (ANGIOTAX) or doxorubicin-based chemotherapy; liposomal doxorubicin in frail patients.
- Am I a candidate for Paclitaxel / nab-paclitaxel, Doxorubicin, Pegylated liposomal doxorubicin, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of ANGIOTAX apply to someone like me?Why: Trial populations differ from individual patients; ask how closely you match.
Later lines
- For my situation (later lines), which of the standard options do you recommend and why?Why: Guideline options include: Gemcitabine, pazopanib; checkpoint inhibitors (nivolumab plus ipilimumab) for cutaneous scalp and face disease, off label or in trials.
- Am I a candidate for Gemcitabine, Pazopanib, Nivolumab or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Immune checkpoint inhibitors, Nivolumab, Ipilimumab, Pazopanib?Why: Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “Multifocal scalp disease escapes even wide surgery and radiotherapy”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Median survival with metastases remains under a year”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
Newly diagnosed? Read the first 60 days with Angiosarcoma, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
10targets
4drugs
8companies
6trials
1Latest papers
topQuery for this cancer: (TITLE:"Angiosarcoma" OR ABSTRACT:"Angiosarcoma" OR TITLE:"Haemangiosarcoma" OR ABSTRACT:"Haemangiosarcoma" OR TITLE:"Lymphangiosarcoma" OR ABSTRACT:"Lymphangiosarcoma" OR TITLE:"Stewart-Treves syndrome lymphoedema-associated angiosarcoma" OR ABSTRACT:"Stewart-Treves syndrome lymphoedema-associated angiosarcoma" OR TITLE:"Radiation-associated angiosarcoma" OR ABSTRACT:"Radiation-associated angiosarcoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Angiosarcoma, not a curated reading list.
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