The first 60 days: Angiosarcoma
Angiosarcoma is an aggressive cancer of the cells that line blood and lymph vessels. It grows as bruise-like patches on the scalp of older people, in breasts treated years earlier with radiotherapy, or inside organs. Surgery and radiotherapy are used where possible, weekly paclitaxel is the most active drug, and immunotherapy helps a minority with the sun-damaged scalp form. Below, week by week, is what OnCo's record of Angiosarcoma 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.
- SurgeonNamed in the standard of care for: Localised cutaneous (scalp, face), Radiation-associated breast angiosarcoma.
- Medical oncologistNamed in the standard of care for: Localised cutaneous (scalp, face), Radiation-associated breast angiosarcoma, Advanced, first line, Later lines.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Localised cutaneous (scalp, face), Radiation-associated breast angiosarcoma.
- Palliative and supportive care teamNamed in the standard of care for: Radiation-associated breast angiosarcoma, Advanced, first line.
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 where feasible plus wide-field radiotherapy; neoadjuvant or definitive weekly paclitaxel with radiotherapy when surgery is not possible.
Total mastectomy with wide skin excision; consider neoadjuvant paclitaxel; re-irradiation is limited by prior dose.
Weekly paclitaxel (ANGIOTAX) or doxorubicin-based chemotherapy; liposomal doxorubicin in frail patients.
Gemcitabine, pazopanib; checkpoint inhibitors (nivolumab plus ipilimumab) for cutaneous scalp and face disease, off label or in trials.
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 MYC amplification, Tumour mutational burden and ultraviolet signature, CD31, ERG and FLI1 endothelial markers, KDR and PLCG1 mutations, PTPRB and PLCG1 in secondary breast tumours), 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 the scalp and face, Radiation-associated angiosarcoma of the breast, Lymphoedema-associated angiosarcoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Localised cutaneous (scalp, face)
- For my situation (localised cutaneous (scalp, face)), which of the standard options do you recommend and why?Guideline options include: Wide excision where feasible plus wide-field radiotherapy; neoadjuvant or definitive weekly paclitaxel with radiotherapy when surgery is not possible.
- 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.
Radiation-associated breast angiosarcoma
- For my situation (radiation-associated breast angiosarcoma), which of the standard options do you recommend and why?Guideline options include: Total mastectomy with wide skin excision; consider neoadjuvant paclitaxel; re-irradiation is limited by prior dose.
- Am I a candidate for Paclitaxel / nab-paclitaxel, Doxorubicin, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced, first line
- For my situation (advanced, first line), which of the standard options do you recommend and why?Guideline options include: Weekly paclitaxel (ANGIOTAX) or doxorubicin-based chemotherapy; liposomal doxorubicin in frail patients.
- Am I a candidate for Paclitaxel / nab-paclitaxel, Doxorubicin, Pegylated liposomal doxorubicin, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of ANGIOTAX apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Later lines
- For my situation (later lines), which of the standard options do you recommend and why?Guideline options include: Gemcitabine, pazopanib; checkpoint inhibitors (nivolumab plus ipilimumab) for cutaneous scalp and face disease, off label or in trials.
- Am I a candidate for Gemcitabine, Pazopanib, Nivolumab 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 Immune checkpoint inhibitors, Nivolumab, Ipilimumab, 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 “Multifocal scalp disease escapes even wide surgery and radiotherapy”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Median survival with metastases remains under a year”. 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.
- Angiosarcoma: the full pageAngiosarcoma is an aggressive cancer of the cells that line blood and lymph vessels. It grows as bruise-like patches on the scalp of older people, in breasts treated years earlier with radiotherapy, or inside organs. Surgery and radiotherapy are used where possible, weekly paclitaxel is the most active drug, and immunotherapy helps a minority with the sun-damaged scalp form.
- 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.