The first 60 days: Myxofibrosarcoma
Myxofibrosarcoma is a sarcoma of older people that grows just beneath the skin of the limbs and sends invisible tails along the tissue planes, so it comes back after surgery more often than any other sarcoma. Wide surgery guided by MRI, usually with radiotherapy, is the treatment; chemotherapy is reserved for spread disease. Below, week by week, is what OnCo's record of Myxofibrosarcoma 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.
- RadiologistNamed in the standard of care for: Localised.
- SurgeonNamed in the standard of care for: Localised, Recurrent local disease, Metastatic.
- Medical oncologistNamed in the standard of care for: Localised, Recurrent local disease, Metastatic.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Localised, Recurrent local disease.
- Palliative and supportive care teamNamed in the standard of care for: Metastatic.
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 planned on contrast-enhanced MRI to include tail-like extensions; preoperative or postoperative radiotherapy for intermediate- and high-grade and recurrent tumours.
Re-resection with radiotherapy if not previously given; amputation only for uncontrollable multiply recurrent disease.
Doxorubicin-based chemotherapy as for other high-grade soft tissue sarcomas; metastasectomy for limited lung 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 FNCLCC grade and grade progression at recurrence, MRI tail sign, TP53, CDKN2A and RB1 alterations, Absence of specific fusion or MDM2 amplification), 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 Low-grade myxofibrosarcoma, Intermediate- and high-grade myxofibrosarcoma, Epithelioid myxofibrosarcoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Localised
- For my situation (localised), which of the standard options do you recommend and why?Guideline options include: Wide resection planned on contrast-enhanced MRI to include tail-like extensions; preoperative or postoperative radiotherapy for intermediate- and high-grade and recurrent tumours.
Recurrent local disease
- For my situation (recurrent local disease), which of the standard options do you recommend and why?Guideline options include: Re-resection with radiotherapy if not previously given; amputation only for uncontrollable multiply recurrent disease.
Metastatic
- For my situation (metastatic), which of the standard options do you recommend and why?Guideline options include: Doxorubicin-based chemotherapy as for other high-grade soft tissue sarcomas; metastasectomy for limited lung disease.
- Am I a candidate for Doxorubicin, Ifosfamide, Gemcitabine or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- 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 “Local recurrence remains the highest of any sarcoma”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “No targetable driver has been found”. 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.
- Myxofibrosarcoma: the full pageMyxofibrosarcoma is a sarcoma of older people that grows just beneath the skin of the limbs and sends invisible tails along the tissue planes, so it comes back after surgery more often than any other sarcoma. Wide surgery guided by MRI, usually with radiotherapy, is the treatment; chemotherapy is reserved for spread disease.
- 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.