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9 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
| Setting | Approach | Guideline |
|---|---|---|
| Asymptomatic, stable, multifocal | Active surveillance with imaging every three to six months; treat only on progression or symptoms. | not mapped |
| Localised | Complete resection; liver transplantation for unresectable liver-limited disease; radiotherapy for bone lesions. | not mapped |
| Progressive or symptomatic | Sirolimus or another mTOR inhibitor first; pazopanib or other anti-angiogenic kinase inhibitors; anthracycline chemotherapy for rapidly progressive disease. | not mapped |