The first 60 days: Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma)
Vascular tumours range from angiosarcoma, an aggressive cancer of blood vessel lining cells, to the slow-growing EHE and the infant tumour KHE. Angiosarcoma responds to paclitaxel and, in the sun-damaged scalp form, to immunotherapy; EHE and KHE depend on growth signals that the mTOR blocker sirolimus quiets, and EHE without symptoms is watched. Below, week by week, is what OnCo's record of Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform 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: Epithelioid haemangioendothelioma.
- SurgeonNamed in the standard of care for: Localised angiosarcoma, Epithelioid haemangioendothelioma.
- Medical oncologistNamed in the standard of care for: Localised angiosarcoma, Advanced angiosarcoma, Kaposiform haemangioendothelioma with Kasabach-Merritt.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Localised angiosarcoma.
- Transplant and cell therapy teamNamed in the standard of care for: Epithelioid haemangioendothelioma.
- Palliative and supportive care teamNamed in the standard of care for: Advanced angiosarcoma.
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.
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.
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 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?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 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?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?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?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?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?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?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?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?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?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 “Angiosarcoma outside the UV-exposed skin remains chemoresistant: immunotherapy combinations and antiangiogenic agents are being tested”. How does that affect my plan?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?Open problems are where trials and second opinions matter most.
Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.
- DART (SWOG S1609): nivolumab plus ipilimumab in rare tumoursPhase 2 · active · NCT02834013More than 50 cohorts of rare solid tumours with no standard therapy: nivolumab plus ipilimumab in a single-arm basket run across the NCI's community network
- MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment of Advanced Soft Tissue SarcomaPhase 2 · recruiting · NCT06277154A Phase II Study Evaluating the Safety and Efficacy of MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment in Patients With Advanced Soft Tissue Sarcoma
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma): the full pageVascular tumours range from angiosarcoma, an aggressive cancer of blood vessel lining cells, to the slow-growing EHE and the infant tumour KHE. Angiosarcoma responds to paclitaxel and, in the sun-damaged scalp form, to immunotherapy; EHE and KHE depend on growth signals that the mTOR blocker sirolimus quiets, and EHE without symptoms is watched.
- 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.
- Rare cancers: Rare cancers are those with fewer than about 6 new cases per 100,000 people per year.
Every term links to the glossary.