Health condition
Angiosarcoma, a rare vascular cancer that needs specialist sarcoma care from day one.
From bruise-like scalp patches in older adults to new nodules on irradiated breast skin, angiosarcoma spreads quickly. Early biopsy and referral to a designated sarcoma centre change the trajectory.
Why trust this guide
- 01
Editorial team
Written by our editorial team from the UK sources listed on each page.
- 02
Sourced from guidance
Checked against NICE, ESMO sarcoma guidance and peer-reviewed sources you can see at the end.
- 03
Current for 2026
Reflects modern UK sarcoma pathways, MYC-driven post-radiotherapy disease and the Angiosarcoma Project.
Key facts
Angiosarcoma at a glance.
The essentials in plain English. What it is, where it appears, and why time to specialist referral matters so much.
-
What it is
A rare, aggressive cancer of the endothelial cells that line blood and lymphatic vessels. Fewer than 1 percent of soft-tissue sarcomas.
-
Cutaneous scalp/face
About half of all cases. Elderly patients, sun-exposed skin, bruise-like patches that spread and become nodular or ulcerating.
-
Breast (post-RT)
A late complication 5 to 10 years after breast conservation surgery and radiotherapy. MYC-amplified and highly aggressive.
-
Stewart-Treves
Angiosarcoma arising in chronic lymphoedema, classically the arm after mastectomy or the leg after filariasis or surgery.
-
Visceral
Liver, heart and spleen. Historical liver angiosarcoma links to vinyl chloride, arsenic and Thorotrast contrast.
-
Prognosis
Poor overall. Five-year survival roughly 30 to 40 percent cutaneous, 20 to 30 percent breast, under 20 percent visceral.
Why this guide matters
Rare, aggressive, and time-critical.
Angiosarcoma often hides as a bruise or a rash for months before the diagnosis lands. Three ideas shape everything that follows.
-
A persistent bruise deserves a biopsy
On the scalp, face or irradiated breast skin of an older adult, a bruise-like patch that will not fade must be biopsied, not observed.
-
Care belongs at a sarcoma centre
Outcomes are meaningfully better when angiosarcoma is planned and delivered by a designated regional sarcoma multidisciplinary team.
-
Modern systemic options exist
Paclitaxel, doxorubicin, propranolol, immunotherapy and targeted agents have all found a role. The landscape is moving.
How the diagnosis is made
From suspicious patch to sarcoma MDT.
The steps a UK dermatologist, breast team or sarcoma centre will usually follow, in order, so you know what to expect.
Phase 1 · Suspicion
Clinical exam and biopsy
Phase 2 · Confirming
Histology, IHC and imaging
Phase 3 · Referral
Staging and sarcoma MDT
- 01
Suspicion
Clinical suspicion
Persistent bruise-like discolouration that does not resolve, a new nodule on irradiated breast skin, or bleeding lesions on the scalp of an older adult.
- 02
Suspicion
Full skin and lymph exam
The visible lesion is often the tip of the iceberg. Multiple satellite areas and lymph nodes are checked carefully.
- 03
Suspicion
Incisional biopsy
Essential. A generous incisional or punch biopsy from a representative area is preferred over shave biopsy to allow proper depth assessment.
- 04
Confirming
Histology and immunohistochemistry
CD31, CD34, ERG, FLI1 and Factor VIII confirm endothelial origin. MYC amplification points to a radiation-associated tumour.
- 05
Confirming
MRI of the local area
Defines soft-tissue extent and depth, which is often far wider than the skin changes suggest, particularly on the scalp.
- 06
Confirming
CT chest, abdomen and pelvis
The lungs are the most common site of spread. Liver and bone are also assessed for metastatic disease.
- 07
Referral
PET-CT staging
FDG PET-CT is often added to detect occult multifocal cutaneous disease and distant metastases before treatment planning.
- 08
Referral
Sarcoma MDT referral
Urgent referral to a designated regional sarcoma centre. Angiosarcoma should not be managed outside a specialist MDT.
Typical timeline: biopsy to sarcoma MDT within two to three weeks on an urgent pathway.
Symptoms
What angiosarcoma actually looks like.
The mix depends on the site. Skin lesions dominate cutaneous and breast disease, while visceral tumours present with organ-specific symptoms and bleeding.
-
Bruise-like patch
A purple or red patch on the scalp or face that looks like a bruise but does not fade over weeks or months.
-
Nodular thickening
Firm nodules and plaques rising within the discoloured area. Often multiple and widely spread across the scalp.
-
Telangiectasia
Fine dilated vessels on the surface. On post-radiotherapy breast skin these may be an early sign of angiosarcoma.
-
Bleeding and ulceration
Late lesions bleed easily, ulcerate and crust. Recurrent bleeding from a scalp bruise in an older adult is a red flag.
-
Post-RT breast changes
A new rash, bruise or nodule on skin previously treated with radiotherapy for breast cancer. Any change deserves biopsy.
-
Chronic swollen limb
In Stewart-Treves the arm after mastectomy or a chronically swollen leg develops firm violaceous nodules within lymphoedema.
-
Visceral symptoms
Nonspecific abdominal pain, anaemia, unexplained bleeding, or cardiac symptoms depending on the primary site.
-
Red flag - urgent referral
Any of the above warrant urgent dermatology or sarcoma referral. Angiosarcoma spreads quickly and time to treatment matters.
Treatment
How angiosarcoma is treated in the UK.
Multimodal, MDT-led, and increasingly systemic. Surgery, radiotherapy, chemotherapy, propranolol, immunotherapy and targeted agents each have a role.
-
Wide local excision
Surgery aiming for clear margins. On the scalp and face true R0 resection is often not achievable because subclinical spread runs far beyond visible lesions.
-
Adjuvant radiotherapy
Wide-field radiotherapy after or before surgery for cutaneous and breast disease. Delivered by a sarcoma or specialist skin team.
-
Neoadjuvant chemotherapy
Weekly paclitaxel is well tolerated and active in cutaneous angiosarcoma. Doxorubicin and ifosfamide are options for deeper soft-tissue disease.
-
Metastatic chemotherapy
Paclitaxel or doxorubicin-based regimens remain the backbone. Response can be meaningful but rarely durable.
-
Propranolol adjunct
Non-selective beta-blocker with evidence in cutaneous angiosarcoma (Chow 2015). Often added to paclitaxel in specialist practice.
-
Immunotherapy
Pembrolizumab and combined ipilimumab-nivolumab are showing promise in cutaneous angiosarcoma, supported by data from the crowd-sourced Angiosarcoma Project.
-
Targeted therapy
Tyrosine-kinase inhibitors pazopanib, regorafenib and sorafenib have limited but real activity. Bevacizumab targets the VEGF pathway.
-
Palliative and supportive care
Bleeding control, wound care, symptom management and early involvement of specialist palliative teams are central to compassionate care.
Prognosis in context
Five-year survival remains humbling, and honest.
Cutaneous scalp and face disease sits at roughly 30 to 40 percent, post-radiotherapy breast angiosarcoma 20 to 30 percent, and visceral disease under 20 percent. Access to a sarcoma centre, clinical trials and early palliative care all shift these numbers, and outliers do well.
What this guide is based on
The sources behind every claim on this page.
UK national guidance, international sarcoma consensus and the peer-reviewed literature current at last review.
Key references
Guidelines and studies we relied on.
A quiet reminder
This guide is for information, not medical advice.
Your sarcoma team knows your scans, histology and history and can tell you which parts apply to you. If in doubt, ask for a second opinion.
-
ESMO-EURACAN-GENTURIS Clinical Practice Guidelines. Soft tissue and visceral sarcomas.
-
NICE. Improving outcomes for people with sarcoma (IOG).
-
British Sarcoma Group. Guidance on management of soft-tissue sarcomas.
-
Chow W et al. Growth attenuation of cutaneous angiosarcoma with propranolol-mediated beta-blockade. JAMA Dermatology, 2015.
-
Angiosarcoma Project (Count Me In). Patient-partnered research programme, 2024 to 2026 updates.
Red flags
When symptoms need urgent attention.
Angiosarcoma often masquerades as a benign skin change. These are the features that should trigger urgent referral, not watchful waiting.
-
Non-resolving scalp bruise
A bruise-like patch on the scalp or face of an older adult that persists for weeks needs urgent dermatology assessment and biopsy.
-
Post-radiotherapy breast lesion
Any new nodule, rash, bruise or telangiectasia on breast skin previously irradiated for cancer must be biopsied to exclude secondary angiosarcoma.
-
Nodule in chronic lymphoedema
Violaceous or firm nodules within a chronically swollen limb raise concern for Stewart-Treves syndrome and require urgent referral.
-
Bleeding cutaneous lesion
A recurrently bleeding scalp or face lesion in the elderly should be treated as suspected angiosarcoma until proven otherwise.
-
Unexplained anaemia and bleeding
Nonspecific but persistent internal bleeding, particularly with a history of vinyl chloride exposure, warrants hepatic imaging.
-
Rapid growth of any soft-tissue mass
Any soft-tissue mass over 5 cm, deep to fascia, or growing rapidly needs urgent sarcoma pathway referral.
-
Cardiac angiosarcoma symptoms
Progressive breathlessness, pericardial effusion or right-sided heart failure in a younger adult may reflect a cardiac primary.
-
Historic Thorotrast exposure
Very rare now, but any adult with documented Thorotrast contrast exposure decades ago should be reviewed for hepatic angiosarcoma risk.
-
Sudden systemic decline
Rapid weight loss, malaise and multi-organ symptoms in known angiosarcoma point to disseminated disease and need urgent oncology review.
Living with it
A rare cancer, but not one to face alone.
Four things make the biggest practical difference. Being in the right centre, hearing about trials, tapping into rare-cancer communities, and bringing palliative care in early.
A quiet reminder
Second opinions are welcome in sarcoma.
Rare cancers reward specialist volume. Asking for a review at another sarcoma centre is normal and often recommended.
- 01 Centre
Insist on a sarcoma MDT
Outcomes are better when angiosarcoma is managed at a designated regional sarcoma centre. It is reasonable to ask for a second opinion.
- 02 Trials
Ask about clinical trials
Immunotherapy and targeted trials are open in the UK. Rare cancer usually means trial-eligible, and referral pathways exist.
- 03 Support
Use rare-cancer networks
Sarcoma UK and the Angiosarcoma Awareness charity offer peer support, information and links to specialist teams.
- 04 Care
Bring palliative care in early
Early involvement of specialist palliative care improves quality of life and, in many cancers, survival. It runs alongside active treatment.
Frequently asked
Everything we get asked about angiosarcoma.
Straight answers on subtypes, diagnosis, treatment and prognosis.
-
What is angiosarcoma?
Angiosarcoma is a rare and aggressive cancer that arises from the endothelial cells lining blood and lymphatic vessels. It accounts for less than 1 percent of soft-tissue sarcomas but has a disproportionate impact because of how quickly it spreads and how difficult it is to remove completely.
-
What are the main types?
The commonest is cutaneous angiosarcoma of the scalp and face in older adults. Others include breast angiosarcoma after radiotherapy for breast cancer, Stewart-Treves syndrome in chronic lymphoedema, deep soft-tissue tumours in the limbs or retroperitoneum, and visceral disease affecting the liver, heart or spleen.
-
How is it diagnosed?
Diagnosis rests on incisional biopsy with immunohistochemistry showing endothelial markers such as CD31, CD34, ERG, FLI1 and Factor VIII. MYC amplification supports a radiation-associated origin. MRI defines local extent and CT or PET-CT stages the disease before referral to a sarcoma MDT.
-
Why is scalp angiosarcoma so difficult to treat?
Because the disease usually spreads far beyond the visible lesion in a subclinical way, a truly clear surgical margin is often impossible on the scalp and face. Wide radiotherapy and systemic therapy are used alongside surgery to try to control the wider field.
-
What treatments are used?
Wide local excision where possible, radiotherapy, and chemotherapy with weekly paclitaxel or doxorubicin-based regimens. Propranolol is often added as an adjunct in cutaneous disease, and immunotherapy with pembrolizumab or ipilimumab-nivolumab is increasingly used, supported by data from the Angiosarcoma Project.
-
What is the outlook?
Prognosis is guarded. Five-year survival is roughly 30 to 40 percent for cutaneous disease, 20 to 30 percent for breast angiosarcoma and under 20 percent for visceral primaries. Care at a specialist sarcoma centre, access to trials and early palliative care support all make a meaningful difference.
Related content
Keep reading.
-
Breast cancer
Primary breast cancer and post-treatment surveillance.
Learn more -
Melanoma
Aggressive skin cancer with overlapping pathways.
Learn more -
Basal cell carcinoma
The commonest skin cancer, for context.
Learn more -
Lymphoma
Another haematological and soft-tissue malignancy.
Learn more -
Mohs micrographic surgery
Related treatment option for skin cancer.
Learn more -
Sentinel lymph node biopsy
Nodal staging used in skin and breast malignancies.
Learn more -
Superficial radiotherapy for skin
Radiotherapy option for selected skin cancers.
Learn more -
Immunotherapy infusion clinic
Where checkpoint inhibitors are delivered.
Learn more -
Breast implant removal (explant)
Relevant if implants complicate surveillance.
Learn more -
Tumour molecular profiling
Genomic testing to guide targeted therapy.
Learn more -
PET-CT scan
Related staging investigation.
Learn more -
Private MRI scan
Related diagnostic test.
Learn more