Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma)
Vascular tumours range from angiosarcoma, an aggressive cancer of blood vessel lining cells, to slow-growing EHE and the infant tumour KHE. Angiosarcoma responds to the chemotherapy paclitaxel and, in the sun-damaged scalp form, to immunotherapy; EHE and KHE are driven by growth signals that the mTOR blocker sirolimus can quiet, and many EHE cases can simply be watched.
Overview
Malignant and intermediate vascular tumours share an endothelial origin but differ sharply in behaviour. Angiosarcoma is a high-grade sarcoma arising in sun-damaged skin of the scalp and face of older adults, in the breast after radiotherapy (with MYC amplification), in lymphoedematous limbs (Stewart-Treves) or in viscera; ultraviolet-signature cutaneous tumours carry a high mutation burden. Epithelioid haemangioendothelioma (EHE) is defined by the WWTR1-CAMTA1 fusion (or YAP1-TFE3 in a minority) that constitutively activates the Hippo pathway effector TAZ; it is multifocal in liver, lung and bone and may stay stable for years. Kaposiform haemangioendothelioma (KHE) is an infantile tumour with lymphatic features that can trigger the Kasabach-Merritt phenomenon, a consumptive coagulopathy. Infantile haemangioma, though benign, is on the same NCI page and is treated with propranolol.
Angiosarcoma is treated with wide resection and radiotherapy when localised; paclitaxel (ANGIOTAX) is the preferred first-line chemotherapy, with doxorubicin and gemcitabine-based regimens as alternatives, and propranolol has been added in some series. Checkpoint inhibitors produce responses particularly in cutaneous head and neck angiosarcoma (DART SWOG S1609 cohort, ipilimumab plus nivolumab), consistent with its UV-driven mutation load. EHE is managed by surveillance when asymptomatic, sirolimus when progressive (mTOR inhibition targets the tumour's dependency on PI3K-mTOR signalling downstream of TAZ), and transplant is considered for isolated hepatic disease. KHE with Kasabach-Merritt is treated with sirolimus, which has replaced vincristine and steroids as first line in many centres.
Mechanistic frontiers are TEAD inhibitors that block the TAZ-TEAD transcriptional complex in EHE, and immunotherapy combinations for angiosarcoma.
State of the art today
- Immunotherapy has entered angiosarcoma via the UV-signature cutaneous form, where high mutation burden predicts response to ipilimumab plus nivolumab.
- EHE has a single fusion driver, WWTR1-CAMTA1, and TEAD inhibitors that block its transcriptional output are the first fusion-directed drugs in trials for the disease.
- Sirolimus repositioned from transplant medicine has become the medical treatment of choice for both EHE and KHE.
- Propranolol turned infantile haemangioma management from steroids and surgery into a safe oral therapy after a chance observation in 2008.
Where it starts and where it drains
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 scalp and face (UV signature) · Radiation-associated breast angiosarcoma (MYC-amplified) · Visceral and cardiac angiosarcoma · Epithelioid haemangioendothelioma (WWTR1-CAMTA1 or YAP1-TFE3) · Kaposiform haemangioendothelioma (infants; Kasabach-Merritt phenomenon)
- 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: Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma
Angiosarcoma accounts for about 1 to 2 percent of soft tissue sarcomas; EHE and KHE are rarer still, with KHE mostly in infants.
- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Where the cases are
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
Wide excision with radiotherapy; margins are often positive because of field spread in the scalp, and neoadjuvant paclitaxel is used to downstage.
Weekly paclitaxel (ANGIOTAX) or doxorubicin-based chemotherapy; gemcitabine-docetaxel, pazopanib; checkpoint inhibitors for cutaneous head and neck disease (DART cohort) or in trials.
Active surveillance if asymptomatic and stable; sirolimus for progressive or symptomatic disease; surgery or liver transplant for isolated hepatic disease.
Sirolimus, with steroids in the acute phase; vincristine as an alternative; platelet transfusion avoided unless bleeding because it feeds the consumptive process.
Subtypes & biomarkers
top- Cutaneous angiosarcoma of scalp and face (UV signature)
- Radiation-associated breast angiosarcoma (MYC-amplified)
- Visceral and cardiac angiosarcoma
- Epithelioid haemangioendothelioma (WWTR1-CAMTA1 or YAP1-TFE3)
- Kaposiform haemangioendothelioma (infants; Kasabach-Merritt phenomenon)
- Infantile haemangioma (benign)
- ERG, CD31, CD34 endothelial markers
- MYC amplification (radiation-associated angiosarcoma)
- WWTR1-CAMTA1 or YAP1-TFE3 fusion (EHE)
- Tumour mutational burden and UV signature (cutaneous angiosarcoma)
- Platelet count and fibrinogen (Kasabach-Merritt in KHE)
Target prevalence in this cancer
- 1940Stewart and Treves describe lymphangiosarcoma after mastectomy
- 1982Weiss and Enzinger define epithelioid haemangioendothelioma
- 2008Propranolol for infantile haemangioma
Léauté-Labrèze and colleagues, NEJM: serendipitous discovery.
- 2008ANGIOTAX: weekly paclitaxel in angiosarcoma
Penel and colleagues, JCO, phase 2.
- 2011WWTR1-CAMTA1 fusion identified in EHE
Tanas and colleagues, Sci Transl Med; Errani and colleagues.
- 2018Checkpoint inhibitors in cutaneous angiosarcoma
Florou and colleagues; later confirmed in the DART SWOG S1609 angiosarcoma cohort (2021).
- 2021EHE international consensus
Surveillance first, sirolimus for progression (ESMO Open).
Open problems, and what is being done about each
Angiosarcoma outside the UV-exposed skin remains chemoresistant: immunotherapy combinations and antiangiogenic agents are being tested.
EHE: which patients will progress, and TEAD inhibitors as the first fusion-directed therapy.
Radiation-associated breast angiosarcoma incidence after breast-conserving therapy: hyperfractionated re-irradiation and surgery are being studied.
Paediatric vascular tumours are so rare that the evidence base is largely case series; registries are the response.
and how the field plans to fix it →What is being done about thisRare cancers and small trialsAvailable now- AI trial matching & clinical decision supportEstablished
- Comprehensive genomic profilingStandard of care
- Dinutuximab (ch14.18) / dinutuximab betaApproved
- Eflornithine (DFMO)Approved
- LarotrectinibApproved
- Limb-salvage surgery and endoprosthetic reconstructionStandard of care
In trials- ACTIONRecruiting
- COG AALL1731Positive
- COG ANBL0032Positive
- DeFiPositive
- Euro Ewing 2012Positive
- Functional (ex vivo) drug testingEmerging
Ideas and roadmaps- A digital second-opinion network answering community oncologists within 72 hours
- A DRUP-style protocol for off-label generic targeted drugs in rare tumours
- A funded expert second opinion for every new high-stakes or rare cancer diagnosis
- A global first-in-human network for academic cancer trials with single ethics review
- A global open trials operating system any hospital can plug into
- A live 'seats available' feed for trial slots, like airline inventory
Background: Basket, umbrella, and platform trials, Centralisation and high-volume centres, FNCLCC grade (soft-tissue sarcoma), Histotype-tailored therapy, INRG staging and risk groups. Also on OnCo: Find a trial · Expert centres.
Trials
topRecruiting now (live from ClinicalTrials.gov)
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Expert centres
topCentres linked to this cancer in OnCo
- via Liver transplantation for cancer (Milan criteria and beyond)
- via Liver transplantation for cancer (Milan criteria and beyond)
- via Immune checkpoint inhibitors
- via Immune checkpoint inhibitors, Nivolumab, Ipilimumab
- via Immune checkpoint inhibitors, Nivolumab, Ipilimumab
- via Immune checkpoint inhibitors, Nivolumab, Ipilimumab
- Geneva University Hospitals (HUG)Geneva, CHvia Immune checkpoint inhibitors, IMRT / IGRT (modern external beam)
- Hunan Cancer HospitalChangsha, CNvia Immune checkpoint inhibitors, IMRT / IGRT (modern external beam)
- IRCCS Humanitas Research HospitalRozzano (Milan), ITvia Immune checkpoint inhibitors, IMRT / IGRT (modern external beam)
- Kyoto University HospitalKyoto, JPvia Immune checkpoint inhibitors, Nivolumab
- via Immune checkpoint inhibitors, IMRT / IGRT (modern external beam)
- via Immune checkpoint inhibitors, IMRT / IGRT (modern external beam)
- via Immune checkpoint inhibitors, IMRT / IGRT (modern external beam)
- Tel Aviv Sourasky Medical CenterTel Aviv, ILvia Immune checkpoint inhibitors, IMRT / IGRT (modern external beam)
- Zhejiang Cancer HospitalHangzhou, CNvia Immune checkpoint inhibitors, IMRT / IGRT (modern external beam)
- Aarhus University HospitalAarhus, DKvia IMRT / IGRT (modern external beam)
- via Nivolumab
- American Society for Radiation OncologyArlington, VA, USvia IMRT / IGRT (modern external beam)
- via Immune checkpoint inhibitors
- via Immune checkpoint inhibitors
- Cedars-Sinai CancerLos Angeles, CA, USvia Immune checkpoint inhibitors
- Centre Antoine LacassagneNice, FRvia IMRT / IGRT (modern external beam)
- Centre hospitalier de l'Université de Montréal (CHUM)Montréal, QC, CAvia Immune checkpoint inhibitors
- Centre Léon BérardLyon, FRvia Immune checkpoint inhibitors
- Centre Oscar LambretLille, FRvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Chinese Society of Clinical OncologyBeijing, CNvia Immune checkpoint inhibitors
- Comprehensive Cancer Center Freiburg (CCCF)Freiburg im Breisgau, DEvia IMRT / IGRT (modern external beam)
- ETOP IBCSG Partners FoundationBern, CHvia Immune checkpoint inhibitors
- European Society for Radiotherapy and OncologyBrussels, BEvia IMRT / IGRT (modern external beam)
- German Hodgkin Study GroupCologne, DEvia Nivolumab
- via Immune checkpoint inhibitors
- GOG FoundationPhiladelphia, PA, USvia Immune checkpoint inhibitors
- Groote Schuur Hospital / University of Cape TownCape Town, ZAvia IMRT / IGRT (modern external beam)
- Guangdong Provincial People's HospitalGuangzhou, CNvia Immune checkpoint inhibitors
- via IMRT / IGRT (modern external beam)
- Hacettepe University Cancer InstituteAnkara, TRvia IMRT / IGRT (modern external beam)
- Hadassah Medical CenterJerusalem, ILvia Immune checkpoint inhibitors
- via IMRT / IGRT (modern external beam)
- Henan Cancer HospitalZhengzhou, CNvia Immune checkpoint inhibitors
- Ho Chi Minh City Oncology HospitalHo Chi Minh City, VNvia IMRT / IGRT (modern external beam)
- Hokkaido University HospitalSapporo, JPvia IMRT / IGRT (modern external beam)
- Hospital Universitario 12 de OctubreMadrid, ESvia Immune checkpoint inhibitors
- Institut BergoniéBordeaux, FRvia IMRT / IGRT (modern external beam)
- Institut National d'Oncologie, RabatRabat, MAvia IMRT / IGRT (modern external beam)
- Institut Salah AzaïezTunis, TNvia IMRT / IGRT (modern external beam)
- Institute of Oncology LjubljanaLjubljana, SIvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via Immune checkpoint inhibitors
- International Association for the Study of Lung CancerDenver, CO, USvia Immune checkpoint inhibitors
- via IMRT / IGRT (modern external beam)
- International Extranodal Lymphoma Study GroupBellinzona, CHvia IMRT / IGRT (modern external beam)
- via Immune checkpoint inhibitors
- Istanbul University Institute of OncologyIstanbul, TRvia IMRT / IGRT (modern external beam)
- Istituto Oncologico Veneto IRCCSPadua, ITvia Immune checkpoint inhibitors
- via IMRT / IGRT (modern external beam)
- Juravinski Cancer Centre / Escarpment Cancer Research InstituteHamilton, ON, CAvia IMRT / IGRT (modern external beam)
- Kenyatta National HospitalNairobi, KEvia IMRT / IGRT (modern external beam)
- Korle Bu Teaching HospitalAccra, GHvia IMRT / IGRT (modern external beam)
- Lagos University Teaching HospitalLagos, NGvia IMRT / IGRT (modern external beam)
- via Nivolumab
- via Immune checkpoint inhibitors
- via IMRT / IGRT (modern external beam)
- Leiden University Medical CenterLeiden, NLvia Immune checkpoint inhibitors
- Ludwig Cancer ResearchNew York, USvia Immune checkpoint inhibitors
- Melanoma Research AllianceWashington, DC, USvia Immune checkpoint inhibitors
- National Cancer Center Hospital EastKashiwa, Chiba, JPvia Nivolumab
- via IMRT / IGRT (modern external beam)
- National Institute of Oncology, HungaryBudapest, HUvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Ocean Road Cancer InstituteDar es Salaam, TZvia IMRT / IGRT (modern external beam)
- via Immune checkpoint inhibitors
- via IMRT / IGRT (modern external beam)
- Parker Institute for Cancer ImmunotherapySan Francisco, USvia Immune checkpoint inhibitors
- Peking University Cancer HospitalBeijing, CNvia Immune checkpoint inhibitors
- Rajiv Gandhi Cancer Institute and Research CentreNew Delhi, INvia IMRT / IGRT (modern external beam)
- Rambam Health Care CampusHaifa, ILvia IMRT / IGRT (modern external beam)
- Rigshospitalet – Copenhagen University HospitalCopenhagen, DKvia IMRT / IGRT (modern external beam)
- Royal Adelaide HospitalAdelaide, AUvia IMRT / IGRT (modern external beam)
- Shanghai Pulmonary HospitalShanghai, CNvia Immune checkpoint inhibitors
- Sheba Medical CenterRamat Gan, ILvia Immune checkpoint inhibitors
- Siriraj Hospital, Mahidol UniversityBangkok, THvia IMRT / IGRT (modern external beam)
- Society for Immunotherapy of CancerMilwaukee, WI, USvia Immune checkpoint inhibitors
- Society of Gynecologic OncologyChicago, IL, USvia Immune checkpoint inhibitors
- SWOG Cancer Research NetworkPortland, OR, USvia Nivolumab
- Taipei Veterans General HospitalTaipei, TWvia Immune checkpoint inhibitors
- Tata Medical Center, KolkataKolkata, INvia IMRT / IGRT (modern external beam)
- Tawam HospitalAl Ain, AEvia IMRT / IGRT (modern external beam)
- The Hospital for Sick Children (SickKids)Toronto, ON, CAvia Immune checkpoint inhibitors
- via IMRT / IGRT (modern external beam)
- via Immune checkpoint inhibitors
- TROG Cancer ResearchNewcastle, NSW, AUvia IMRT / IGRT (modern external beam)
- UMC Utrecht Cancer CenterUtrecht, NLvia IMRT / IGRT (modern external beam)
- via Immune checkpoint inhibitors
- via Immune checkpoint inhibitors
- via Immune checkpoint inhibitors
- via Immune checkpoint inhibitors
- University of Malaya Medical CentreKuala Lumpur, MYvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Velindre Cancer CentreCardiff, GBvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- West Japan Oncology GroupOsaka, JPvia Immune checkpoint inhibitors
- via Immune checkpoint inhibitors
Questions to ask
topQuestions to ask your oncologist about Vascular tumours
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 ERG, CD31, CD34 endothelial markers, MYC amplification, WWTR1-CAMTA1 or YAP1-TFE3 fusion, Tumour mutational burden and UV signature, Platelet count and fibrinogen), 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 scalp and face, Radiation-associated breast angiosarcoma, Visceral and cardiac angiosarcoma.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Localised angiosarcoma
- For my situation (localised angiosarcoma), which of the standard options do you recommend and why?Why: Guideline options include: Wide excision with radiotherapy; margins are often positive because of field spread in the scalp, and neoadjuvant paclitaxel is used to downstage.
- 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.
Advanced angiosarcoma
- For my situation (advanced angiosarcoma), which of the standard options do you recommend and why?Why: Guideline options include: Weekly paclitaxel (ANGIOTAX) or doxorubicin-based chemotherapy; gemcitabine-docetaxel, pazopanib; checkpoint inhibitors for cutaneous head and neck disease (DART cohort) or in trials.
- Am I a candidate for Paclitaxel / nab-paclitaxel, Doxorubicin, Pazopanib or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Epithelioid haemangioendothelioma
- For my situation (epithelioid haemangioendothelioma), which of the standard options do you recommend and why?Why: Guideline options include: Active surveillance if asymptomatic and stable; sirolimus for progressive or symptomatic disease; surgery or liver transplant for isolated hepatic disease.
Kaposiform haemangioendothelioma with Kasabach-Merritt
- For my situation (kaposiform haemangioendothelioma with kasabach-merritt), which of the standard options do you recommend and why?Why: Guideline options include: Sirolimus, with steroids in the acute phase; vincristine as an alternative; platelet transfusion avoided unless bleeding because it feeds the consumptive process.
- Am I a candidate for Vincristine, 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, Paclitaxel / nab-paclitaxel, 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 “Angiosarcoma outside the UV-exposed skin remains chemoresistant: immunotherapy combinations and antiangiogenic agents are being tested”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “EHE: which patients will progress, and TEAD inhibitors as the first fusion-directed therapy”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
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Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
8targets
4drugs
6companies
3pathways
4terms
1bottlenecks
1Latest papers
topQuery for this cancer: (TITLE:"Vascular tumours" OR ABSTRACT:"Vascular tumours" OR TITLE:"angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma" OR ABSTRACT:"angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma" OR TITLE:"Childhood vascular tumours" OR ABSTRACT:"Childhood vascular tumours" OR TITLE:"Angiosarcoma" OR ABSTRACT:"Angiosarcoma" OR TITLE:"EHE" OR ABSTRACT:"EHE" OR TITLE:"KHE" OR ABSTRACT:"KHE") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), not a curated reading list.
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