Epithelioid haemangioendothelioma
Epithelioid haemangioendothelioma is a rare vascular cancer driven by a fusion gene, usually WWTR1-CAMTA1, that behaves unpredictably: some tumours sit unchanged for years while others spread quickly. Stable disease is watched, localised tumours are removed, liver-only disease can be transplanted, and mTOR blockers such as sirolimus are the most used drugs when treatment is needed.
Overview
Epithelioid haemangioendothelioma is a malignant vascular tumour of intermediate to high grade defined by the WWTR1-CAMTA1 fusion in about nine in ten cases and the YAP1-TFE3 fusion in most of the rest; both fusions hijack the Hippo pathway effectors TAZ and YAP. It presents as single or multifocal nodules in the liver, lungs, bone or soft tissue, often with pleural or peritoneal involvement, and is frequently found incidentally. Effusions, pain and weight loss mark the aggressive phenotype, while multifocal liver and lung disease is often indolent.
Because the natural history is so variable, the first decision is whether to treat at all: asymptomatic, stable, multifocal disease is placed under active surveillance with serial imaging. Localised tumours are resected, and hepatic epithelioid haemangioendothelioma is one of the few metastatic-appearing cancers for which liver transplantation is accepted, with good long-term survival in registry series. Radiotherapy palliates bone and painful lesions.
Systemic therapy is used for progressive or symptomatic disease. Sirolimus produced disease stabilisation and some responses in an Italian Sarcoma Group series and prospective study, and mTOR inhibition is the most widely used first option; anti-angiogenic kinase inhibitors such as pazopanib and the MEK inhibitor trametinib (SARC033) have modest activity, and anthracycline-based chemotherapy is reserved for rapidly progressive disease. Patient groups such as the EHE Foundation have funded fusion-directed drug discovery, including TAZ-CAMTA1 degraders.
State of the art
- Active surveillance is standard for stable disease because many tumours do not progress for years.
- Sirolimus is the most used drug, and TAZ-CAMTA1-directed agents are in preclinical development.
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.
- Liver transplantation offers long survival in hepatic disease despite multifocality.
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 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: Everolimus
Avoid grapefruit. Live vaccines are contraindicated.
- 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:DoxorubicinEverolimusPaclitaxel / nab-paclitaxelPazopanib·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 compartmentWWTR1-CAMTA1 epithelioid haemangioendothelioma (about 90 percent) · YAP1-TFE3 epithelioid haemangioendothelioma (younger, more vasoformative) · Hepatic epithelioid haemangioendothelioma (transplant candidate) · Pulmonary and pleural epithelioid haemangioendothelioma (effusions, poorer outlook) · Bone and soft tissue epithelioid haemangioendothelioma
- 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, Angiosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Myxofibrosarcoma, Alveolar soft part sarcoma, Perivascular epithelioid cell tumour (PEComa), Malignant peripheral nerve sheath tumour (MPNST), Retroperitoneal sarcoma, Soft tissue sarcoma of the extremity (localised and advanced)
Roughly one case per million people a year, in adults of any age, arising in the liver, lungs, bone and soft tissue and often multifocal at diagnosis; its course ranges from years of stability to rapid progression, and no drug is approved.
- MRIStandard of care
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
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.
Active surveillance with imaging every three to six months; treat only on progression or symptoms.
Complete resection; liver transplantation for unresectable liver-limited disease; radiotherapy for bone lesions.
Sirolimus or another mTOR inhibitor first; pazopanib or other anti-angiogenic kinase inhibitors; anthracycline chemotherapy for rapidly progressive disease.
Subtypes & biomarkers
top- WWTR1-CAMTA1 epithelioid haemangioendothelioma (about 90 percent)
- YAP1-TFE3 epithelioid haemangioendothelioma (younger, more vasoformative)
- Hepatic epithelioid haemangioendothelioma (transplant candidate)
- Pulmonary and pleural epithelioid haemangioendothelioma (effusions, poorer outlook)
- Bone and soft tissue epithelioid haemangioendothelioma
- WWTR1-CAMTA1 fusion (CAMTA1 immunohistochemistry)
- YAP1-TFE3 fusion (TFE3 immunohistochemistry)
- Pleural effusion, serosal involvement and pain (aggressive phenotype)
- Mitotic count and size (risk stratification)
How often this target appears
- 1982Weiss and Enzinger describe epithelioid haemangioendothelioma
- 2011WWTR1-CAMTA1 fusion identified as the defining alteration
- 2013YAP1-TFE3 fusion found in a second subset
- 2016Sirolimus activity reported in progressive epithelioid haemangioendothelioma
- 2021SARC033: trametinib shows modest activity
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 6 changes by month →- 2026-09-17This recordEpithelioid haemangioendotheliomaFacts on this page last checked
When this page itself was last checked or edited.
- 2021MilestoneMEK1/2SARC033: trametinib shows modest activity
A milestone in how this cancer is treated.
- 2016MilestonemTORSirolimus activity reported in progressive epithelioid haemangioendothelioma
A milestone in how this cancer is treated.
- 2013MilestoneEpithelioid haemangioendotheliomaYAP1-TFE3 fusion found in a second subset
A milestone in how this cancer is treated.
- 2011MilestoneEpithelioid haemangioendotheliomaWWTR1-CAMTA1 fusion identified as the defining alteration
A milestone in how this cancer is treated.
- 1982MilestoneEpithelioid haemangioendotheliomaWeiss and Enzinger describe epithelioid haemangioendothelioma
A milestone in how this cancer is treated.
What is in development for Epithelioid haemangioendothelioma, drawn from the whole corpus: 1 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.
Targets under investigation · 1
Open problems and what is being done
No approved drug and no randomised trial.
Predicting which tumours will progress is unreliable.
The fusion oncoprotein is a transcription factor with no direct inhibitor yet.
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.
Expert centres
topExpert centres
Seoul · hospital | South Korea | none recorded | 0 | 1,177 | 18,820 | none recorded | #4 |
Rochester, MN · hospital | United States | 0 | 4,511 | 44,748 | #5 | ||
Utrecht · cancer center | Netherlands | none recorded | 0 | 1,422 | 20,323 | - | |
Tianjin · cancer center | China | none recorded | 0 | 1,219 | 11,455 | - | |
Hangzhou · cancer center | China | none recorded | 0 | 1,219 | 17,635 | - | |
Rozzano (Milan) · hospital | Italy | none recorded | 0 | 1,031 | 10,720 | - | |
Changsha · cancer center | China | none recorded | 0 | 930 | 14,477 | - | |
Jinan · cancer center | China | none recorded | 0 | 920 | 7,804 | - | |
Rotterdam · cancer center | Netherlands | none recorded | 0 | 852 | 12,180 | - | |
Dresden · cancer center | Germany | none recorded | 0 | 728 | 7,984 | - | |
| United Kingdom | none recorded | 0 | 693 | 7,168 | - | ||
Aarhus · hospital | Denmark | none recorded | 0 | 624 | 4,497 | - | |
Goyang · cancer center | South Korea | none recorded | 0 | 573 | 9,401 | - | |
Freiburg im Breisgau · cancer center | Germany | none recorded | 0 | 549 | 5,261 | - | |
Shanghai · hospital | China | none recorded | 0 | 464 | 6,795 | - |
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 Epithelioid haemangioendothelioma 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 Epithelioid haemangioendothelioma
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 WWTR1-CAMTA1 fusion, YAP1-TFE3 fusion, Pleural effusion, serosal involvement and pain, Mitotic count and size), 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 WWTR1-CAMTA1 epithelioid haemangioendothelioma, YAP1-TFE3 epithelioid haemangioendothelioma, Hepatic epithelioid haemangioendothelioma.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Asymptomatic, stable, multifocal
- For my situation (asymptomatic, stable, multifocal), which of the standard options do you recommend and why?Why: Guideline options include: Active surveillance with imaging every three to six months; treat only on progression or symptoms.
Localised
- For my situation (localised), which of the standard options do you recommend and why?Why: Guideline options include: Complete resection; liver transplantation for unresectable liver-limited disease; radiotherapy for bone lesions.
Progressive or symptomatic
- For my situation (progressive or symptomatic), which of the standard options do you recommend and why?Why: Guideline options include: Sirolimus or another mTOR inhibitor first; pazopanib or other anti-angiogenic kinase inhibitors; anthracycline chemotherapy for rapidly progressive disease.
- Am I a candidate for Everolimus, Sirolimus protein-bound particles, Pazopanib 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 mTOR, 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 “No approved drug and no randomised trial”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Predicting which tumours will progress is unreliable”. 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 Epithelioid haemangioendothelioma, 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
11targets
6drugs
5companies
2Latest papers
topQuery for this cancer: (TITLE:"Epithelioid haemangioendothelioma" OR ABSTRACT:"Epithelioid haemangioendothelioma" OR TITLE:"EHE" OR ABSTRACT:"EHE" OR TITLE:"WWTR1-CAMTA1 sarcoma" OR ABSTRACT:"WWTR1-CAMTA1 sarcoma" OR TITLE:"YAP1-TFE3 haemangioendothelioma" OR ABSTRACT:"YAP1-TFE3 haemangioendothelioma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Epithelioid haemangioendothelioma, not a curated reading list.
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