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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.
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.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
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.
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.
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Adamantinoma of bone, Dedifferentiated chordoma, Poorly differentiated chordoma (SMARCB1-deficient), Desmoplastic small round cell tumour, 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)
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
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.
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Sirolimus is the first-choice systemic therapy for progressing EHE, ideally started before pleural or peritoneal effusions develop.
The observation-first approach and the sequencing of sirolimus before other systemic options on the EHE page come from this consensus.
CAMTA1 immunohistochemistry and fusion testing confirm the diagnosis, and the TAZ-CAMTA1 fusion protein's dependence on the Hippo pathway is guiding drug development.
Query 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.
The targets of this cancer's medicines and the ones linked to it directly.
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.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Avoid grapefruit. Live vaccines are contraindicated.
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
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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.
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