The first 60 days: Ewing sarcoma
Ewing sarcoma is a bone and soft-tissue cancer of teenagers driven by a single fusion gene, EWSR1-FLI1. Intensive chemotherapy with surgery or radiation cures most localised cases; disease that has spread at diagnosis, and relapse, remain hard to treat, and no drug against the fusion protein itself has yet succeeded. Below, week by week, is what OnCo's record of Ewing sarcoma 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.
- Medical oncologistNamed in the standard of care for: Localised, Metastatic (lung only), Metastatic (bone/marrow), Relapsed.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Localised, Metastatic (lung only), Relapsed.
- Transplant and cell therapy teamNamed in the standard of care for: Localised, Metastatic (lung only), Metastatic (bone/marrow), Relapsed.
- Palliative and supportive care teamNamed in the standard of care for: Localised, Metastatic (lung only), Metastatic (bone/marrow).
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.
Interval-compressed VDC/IE ×14 cycles (AEWS0031 / Euro Ewing 2012) with surgery ± radiotherapy (or definitive RT 55.8 Gy) after 6 induction cycles.
Same chemotherapy plus whole-lung irradiation; busulfan-melphalan high-dose therapy in selected patients (Euro-EWING 99 R2pulm equivocal).
Chemotherapy with palliative-intent local therapy; survival <20%; trials strongly preferred.
High-dose ifosfamide (rEECur), irinotecan-temozolomide, topotecan-cyclophosphamide, gemcitabine-docetaxel; local therapy; 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 EWSR1-FLI1 / EWSR1-ERG fusion, CD99 membranous staining, NKX2-2, Metastatic status, Histologic response to induction, STAG2 loss, TP53 mutation, CDKN2A deletion), 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 Skeletal Ewing sarcoma, Extraosseous Ewing sarcoma, EWSR1-FLI1 vs EWSR1-ERG fusion.
- 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: Interval-compressed VDC/IE ×14 cycles (AEWS0031 / Euro Ewing 2012) with surgery ± radiotherapy (or definitive RT 55.8 Gy) after 6 induction cycles.
- Am I a candidate for Vincristine, Doxorubicin, Cyclophosphamide or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Metastatic (lung only)
- For my situation (metastatic (lung only)), which of the standard options do you recommend and why?Guideline options include: Same chemotherapy plus whole-lung irradiation; busulfan-melphalan high-dose therapy in selected patients (Euro-EWING 99 R2pulm equivocal).
- Am I a candidate for Vincristine, Doxorubicin, Cyclophosphamide or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Metastatic (bone/marrow)
- For my situation (metastatic (bone/marrow)), which of the standard options do you recommend and why?Guideline options include: Chemotherapy with palliative-intent local therapy; survival <20%; trials strongly preferred.
- Am I a candidate for Vincristine, Doxorubicin, Cyclophosphamide, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Relapsed
- For my situation (relapsed), which of the standard options do you recommend and why?Guideline options include: High-dose ifosfamide (rEECur), irinotecan-temozolomide, topotecan-cyclophosphamide, gemcitabine-docetaxel; local therapy; trials.
- Am I a candidate for Ifosfamide, Irinotecan (and liposomal irinotecan), Temozolomide 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 Lurbinectedin, Ifosfamide, CAR-T cell therapy, KC1036?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 “Metastatic-to-bone and relapsed disease: survival under 20-30%”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “EWSR1-FLI1 remains undrugged after 30 years”. 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.
- A Study of KC1036 in Adolescents With Advanced Ewing SarcomaPhase 2 · recruiting · NCT06564272A Phase II Study to Evaluate the Safety and Efficacy of KC1036 in Adolescents Aged 12 and Above With Advanced Ewing Sarcoma
- CAMPFIRE: A Study of Abemaciclib (LY2835219) in Participants With Ewing's SarcomaPhase 2 · active · NCT05440786A Randomized, Open-Label, Phase 2 Study Evaluating Abemaciclib in Combination With Irinotecan and Temozolomide in Participants With Relapsed or Refractory Ewing's Sarcoma
- Study Of Palbociclib Combined With Chemotherapy In Pediatric Patients With Recurrent/Refractory Solid TumorsPhase 2 · active · NCT03709680PHASE 1/2 STUDY TO EVALUATE PALBOCICLIB (IBRANCE®) IN COMBINATION WITH IRINOTECAN AND TEMOZOLOMIDE OR IN COMBINATION WITH TOPOTECAN AND CYCLOPHOSPHAMIDE IN PEDIATRIC PATIENTS WITH RECURRENT OR REFRACTORY SOLID TUMORS
- Study of Lurbinectedin Monotherapy in Pediatric and Young Adult Participants With Relapsed/Refractory Ewing SarcomaPhase 1/2 · recruiting · NCT05734066A Phase 1/2, Open-label Study to Evaluate the Safety, Tolerability, Pharmacokinetics (PK), Recommended Phase 2 Dose (RP2D), and Efficacy of Lurbinectedin Monotherapy in Pediatric Participants With Previously Treated Solid Tumors Followed by Expansion to Assess Efficacy and Safety in Pediatric and Young Adult Participants With Relapsed/Refractory Ewing Sarcoma.
- Substudy 01A: Zilovertamab Vedotin in Pediatric and Young Adult Participants With Hematologic Malignancies or Solid Tumors (MK-9999-01A/LIGHTBEAM-U01)Phase 1/2 · recruiting · NCT06395103LIGHTBEAM-U01 Substudy 01A: A Phase 1/2 Substudy to Evaluate the Safety and Efficacy of Zilovertamab Vedotin in Pediatric and Young Adult Participants With Hematologic Malignancies or Solid Tumors
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Ewing sarcoma: the full pageEwing sarcoma is a bone and soft-tissue cancer of teenagers driven by a single fusion gene, EWSR1-FLI1. Intensive chemotherapy with surgery or radiation cures most localised cases; disease that has spread at diagnosis, and relapse, remain hard to treat, and no drug against the fusion protein itself has yet succeeded.
- 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.
- Gene fusion: A gene fusion is two genes broken and joined together, creating a hybrid protein that can drive cancer.
- Adolescent and young adult (AYA) oncology: Cancer in people aged 15-39, about 90,000 US cases a year, with a distinct mix of cancers, slower survival improvement than children or older adults, and specific needs: fertility, education and work, psychosocial support and trial access.
Every term links to the glossary.