The first 60 days: Undifferentiated pleomorphic sarcoma (UPS)
Undifferentiated pleomorphic sarcoma is the diagnosis for a high-grade soft tissue sarcoma whose cells show no recognisable line of differentiation. It is treated with surgery and radiotherapy, with doxorubicin-based chemotherapy for high-risk or spread disease, and it is one of the few sarcomas in which the immunotherapy drug pembrolizumab has clear activity. Below, week by week, is what OnCo's record of Undifferentiated pleomorphic sarcoma (UPS) 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, limb or trunk.
- Medical oncologistNamed in the standard of care for: Localised, limb or trunk, Advanced, first line, Later lines.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Localised, limb or trunk.
- Palliative and supportive care teamNamed in the standard of care for: Localised, limb or trunk, 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 resection with preoperative or postoperative radiotherapy; neoadjuvant anthracycline-ifosfamide for large, deep, high-grade tumours in fit patients (ISG-STS 1001).
Doxorubicin alone or doxorubicin plus ifosfamide (EORTC 62012) when response matters.
Gemcitabine-docetaxel, trabectedin, pazopanib; pembrolizumab in trials or off label after SARC028.
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 FNCLCC grade, size and depth, Exclusion of specific differentiation by immunohistochemistry and molecular testing, TP53, RB1, CDKN2A and ATRX alterations, Tertiary lymphoid structures and B-cell-rich immune class), 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 Undifferentiated pleomorphic sarcoma of the limbs, Undifferentiated pleomorphic sarcoma of the trunk and retroperitoneum, Radiation-associated undifferentiated sarcoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Localised, limb or trunk
- For my situation (localised, limb or trunk), which of the standard options do you recommend and why?Guideline options include: Wide resection with preoperative or postoperative radiotherapy; neoadjuvant anthracycline-ifosfamide for large, deep, high-grade tumours in fit patients (ISG-STS 1001).
- Am I a candidate for Doxorubicin, Ifosfamide, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of ISG-STS 1001 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Advanced, first line
- For my situation (advanced, first line), which of the standard options do you recommend and why?Guideline options include: Doxorubicin alone or doxorubicin plus ifosfamide (EORTC 62012) when response matters.
- Am I a candidate for Doxorubicin, Ifosfamide, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of EORTC 62012 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-docetaxel, trabectedin, pazopanib; pembrolizumab in trials or off label after SARC028.
- Am I a candidate for Gemcitabine, Docetaxel, Trabectedin 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 Pembrolizumab, Immune checkpoint inhibitors, ISG-STS 1001?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 molecular driver to target; the diagnosis is defined by what the tumour is not”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “A third of high-grade limb tumours still metastasise to the lungs”. 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.
- Undifferentiated pleomorphic sarcoma (UPS): the full pageUndifferentiated pleomorphic sarcoma is the diagnosis for a high-grade soft tissue sarcoma whose cells show no recognisable line of differentiation. It is treated with surgery and radiotherapy, with doxorubicin-based chemotherapy for high-risk or spread disease, and it is one of the few sarcomas in which the immunotherapy drug pembrolizumab has clear activity.
- 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.
- FNCLCC grade (soft-tissue sarcoma): The FNCLCC grade is a 1-to-3 score for soft-tissue sarcomas based on how abnormal, how fast-dividing, and how much dead tissue the tumour shows; grade drives whether chemotherapy is considered.
Every term links to the glossary.