# Angiosarcoma

Source: https://onco.cc/cancers/angiosarcoma/  
OnCo record `angiosarcoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## 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.

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: Haemangiosarcoma; Lymphangiosarcoma; Stewart-Treves syndrome (lymphoedema-associated angiosarcoma); Radiation-associated angiosarcoma
- Tags: subtype-page
- 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

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/angiosarcoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/angiosarcoma/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/angiosarcoma/#what-it-is [5 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/angiosarcoma/#finding-it [5 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/angiosarcoma/#treating-it [4 settings, 4 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/angiosarcoma/#evidence [4 trials, 2 key papers, 6 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/angiosarcoma/#science [11 targets]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/angiosarcoma/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/angiosarcoma/#living-with-it [18 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/angiosarcoma/coming/ [9 medicines, 4 trials, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/angiosarcoma/data/ [46 connected records]

## Standard of care

- Localised cutaneous (scalp, face): Wide excision where feasible plus wide-field radiotherapy; neoadjuvant or definitive weekly paclitaxel with radiotherapy when surgery is not possible. ([IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/))
- Radiation-associated breast angiosarcoma: Total mastectomy with wide skin excision; consider neoadjuvant paclitaxel; re-irradiation is limited by prior dose. ([Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/))
- Advanced, first line: Weekly paclitaxel (ANGIOTAX) or doxorubicin-based chemotherapy; liposomal doxorubicin in frail patients. ([Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/), [ANGIOTAX](https://onco.cc/trials/angiotax/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Pegylated liposomal doxorubicin](https://onco.cc/drugs/pegylated-liposomal-doxorubicin/))
- Later lines: Gemcitabine, pazopanib; checkpoint inhibitors (nivolumab plus ipilimumab) for cutaneous scalp and face disease, off label or in trials. ([Gemcitabine](https://onco.cc/drugs/gemcitabine/), [Pazopanib](https://onco.cc/drugs/pazopanib/), [Nivolumab](https://onco.cc/drugs/nivolumab/), [Ipilimumab](https://onco.cc/drugs/ipilimumab/), [Immune checkpoint inhibitors](https://onco.cc/technologies/checkpoint-inhibitor/))

## 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.

## Open problems

- 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.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Angiosarcoma
- Wikipedia: https://en.wikipedia.org/wiki/Angiosarcoma

## Connected records

- cancers: [Epithelioid haemangioendothelioma](https://onco.cc/cancers/epithelioid-haemangioendothelioma/), [Kaposi sarcoma](https://onco.cc/cancers/kaposi-sarcoma/), [Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma)](https://onco.cc/cancers/vascular-tumours/)
- drugs: [Bevacizumab](https://onco.cc/drugs/bevacizumab/), [Carotuximab](https://onco.cc/drugs/carotuximab/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Gemcitabine](https://onco.cc/drugs/gemcitabine/), [Ipilimumab](https://onco.cc/drugs/ipilimumab/), [Nivolumab](https://onco.cc/drugs/nivolumab/), [Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/), [Pazopanib](https://onco.cc/drugs/pazopanib/), [Pegylated liposomal doxorubicin](https://onco.cc/drugs/pegylated-liposomal-doxorubicin/)
- trials: [ANGIOTAX](https://onco.cc/trials/angiotax/), [Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma](https://onco.cc/trials/nct00112463/), [Intratumoral Vusolimogene Oderparepvec (VO) in Combination With Pembrolizumab for Angiosarcoma](https://onco.cc/trials/nct06898970/), [TAPPAS](https://onco.cc/trials/tappas/)
- key papers: [Angiosarcoma (review)](https://onco.cc/key-papers/paper-young-angiosarcoma-review-lancet-oncol-2010/), [ANGIOTAX: phase 2 trial of weekly paclitaxel for unresectable angiosarcoma](https://onco.cc/key-papers/paper-angiotax-paclitaxel-angiosarcoma-penel-jco-2008/)
- technologies: [Immune checkpoint inhibitors](https://onco.cc/technologies/checkpoint-inhibitor/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/)
- people: [Robin L. Jones](https://onco.cc/people/robin-jones/)
- terms: [Sarcoma in the radiotherapy field, and angiosarcoma of the treated breast](https://onco.cc/terms/second-primary-sarcoma-in-the-treated-field/)
- companies: [French Sarcoma Group](https://onco.cc/companies/french-sarcoma-group/)
- targets: [ABCC4](https://onco.cc/targets/abcc4/), [ANK1](https://onco.cc/targets/ank1/), [ERG](https://onco.cc/targets/erg/), [PLCG1](https://onco.cc/targets/plcg1/), [PTPRB](https://onco.cc/targets/ptprb/), [RNF213](https://onco.cc/targets/rnf213/), [ZBTB48](https://onco.cc/targets/zbtb48/)

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JSON: https://onco.cc/api/v1/entities/angiosarcoma.json