The first 60 days: 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. Below, week by week, is what OnCo's record of Epithelioid haemangioendothelioma says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Asymptomatic, stable, multifocal.
- RadiologistNamed in the standard of care for: Asymptomatic, stable, multifocal.
- SurgeonNamed in the standard of care for: Asymptomatic, stable, multifocal, Localised.
- Medical oncologistNamed in the standard of care for: Localised, Progressive or symptomatic.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Localised.
- Transplant and cell therapy teamNamed in the standard of care for: Localised.
- Palliative and supportive care teamNamed in the standard of care for: Progressive or symptomatic.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
Complete resection; liver transplantation for unresectable liver-limited disease; radiotherapy for bone lesions.
Active surveillance with imaging every three to six months; treat only on progression or symptoms.
Sirolimus or another mTOR inhibitor first; pazopanib or other anti-angiogenic kinase inhibitors; anthracycline chemotherapy for rapidly progressive disease.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?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?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?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?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?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?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?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?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?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?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?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?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?Open problems are where trials and second opinions matter most.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Epithelioid haemangioendothelioma: the full pageEpithelioid 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.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.