The first 60 days: Sarcomas (soft tissue, bone, GIST)
Sarcomas are dozens of rare cancers of bone and connective tissue. GIST was the first solid tumour cured-in-practice by a targeted pill; synovial sarcoma got the first TCR-T therapy. Below, week by week, is what OnCo's record of Sarcomas (soft tissue, bone, GIST) 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 STS, Localised extremity/trunk soft-tissue sarcoma, low grade, Localised high-risk soft-tissue sarcoma (grade 3, >5 cm, deep), GIST, localised and 5 more.
- Medical oncologistNamed in the standard of care for: Localised STS, Advanced, Localised extremity/trunk soft-tissue sarcoma, low grade, Localised high-risk soft-tissue sarcoma (grade 3, >5 cm, deep) and 8 more.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Localised STS, Localised extremity/trunk soft-tissue sarcoma, low grade, Localised high-risk soft-tissue sarcoma (grade 3, >5 cm, deep), Ewing sarcoma.
- Transplant and cell therapy teamNamed in the standard of care for: Advanced, Advanced soft-tissue sarcoma, later lines.
- Palliative and supportive care teamNamed in the standard of care for: Localised high-risk soft-tissue sarcoma (grade 3, >5 cm, deep), Advanced soft-tissue sarcoma, first line, Osteosarcoma, Ewing sarcoma.
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 + radiation; neoadjuvant chemotherapy for high-risk.
- 2.Localised extremity/trunk soft-tissue sarcoma, low gradeNCCN category Category 1 (surgery ± RT), NCCN Soft Tissue Sarcoma 2026
Wide resection (limb-salvage) ± radiotherapy for margins or size >5 cm; observation thereafter.
Resection; adjuvant imatinib 3 years for high risk (SSGXVIII), longer under study; none for PDGFRA D842V or SDH-deficient (imatinib-insensitive).
- 4.Localised high-risk soft-tissue sarcoma (grade 3, >5 cm, deep)NCCN category Category 2A (neoadjuvant chemotherapy for high-risk), ESMO-MCBS A (ISG-STS 1001), NCCN 2026 / ESMO 2021
Neoadjuvant anthracycline-ifosfamide × 3 (ISG-STS 1001) ± preoperative radiotherapy, then wide resection; regional hyperthermia with chemotherapy where available (EORTC 62961).
Doxorubicin ± ifosfamide; subtype-directed: imatinib, afami-cel, larotrectinib (tazemetostat was withdrawn in March 2026).
Doxorubicin 75 mg/m2 (single agent) or doxorubicin-ifosfamide for symptomatic/rapid disease (EORTC 62012: PFS but not OS benefit); histotype exceptions: trabectedin or gemcitabine-docetaxel for leiomyosarcoma, paclitaxel for angiosarcoma. Adding olaratumab to doxorubicin gave no survival benefit (ANNOUNCE).
Imatinib 400 mg (800 mg for exon 9) → sunitinib → regorafenib → ripretinib (INVICTUS); avapritinib for PDGFRA D842V; ctDNA KIT genotyping to choose ripretinib vs sunitinib second line (INSIGHT); surgery for oligoprogression.
Neoadjuvant MAP (methotrexate, doxorubicin, cisplatin) → limb-salvage resection → adjuvant MAP; no benefit from adding ifosfamide-etoposide or interferon (EURAMOS-1); metastatic/relapsed: surgery of lung metastases, regorafenib/cabozantinib.
Interval-compressed VDC/IE (INT-0091, AEWS0031, Euro Ewing 2012) with surgery and/or radiotherapy for local control; high-dose busulfan-melphalan for selected high-risk (Euro-EWING 99 R2); relapse: irinotecan-temozolomide, cabozantinib, trials.
Active surveillance first (many regress); nirogacestat (DeFi) for progressing symptomatic disease; sorafenib alternative; surgery only for select sites; cryoablation for extra-abdominal tumours.
Surgery for localised disease; vimseltinib (MOTION) or pexidartinib (REMS for hepatotoxicity) for diffuse disease not amenable to surgery.
Trabectedin (L-sarcomas), eribulin (liposarcoma), pazopanib (non-adipocytic), gemcitabine-docetaxel, dacarbazine; pembrolizumab for alveolar soft-part sarcoma or UPS; larotrectinib for NTRK fusion; afami-cel or lete-cel for MAGE-A4/NY-ESO-1+ synovial sarcoma and MRCLS.
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 Histologic subtype, KIT/PDGFRA, MAGE-A4 + HLA-A*02, NTRK fusions, INI1 loss), 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 Soft-tissue sarcoma: liposarcoma, leiomyosarcoma, undifferentiated pleomorphic sarcoma, synovial sarcoma, myxofibrosarcoma, angiosarcoma, MPNST, rhabdomyosarcoma, Bone sarcoma: osteosarcoma, Ewing sarcoma, chondrosarcoma, chordoma, Gastrointestinal stromal tumour.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Localised STS
- For my situation (localised sts), which of the standard options do you recommend and why?Guideline options include: Wide excision + radiation; neoadjuvant chemotherapy for high-risk.
Advanced
- For my situation (advanced), which of the standard options do you recommend and why?Guideline options include: Doxorubicin ± ifosfamide; subtype-directed: imatinib, afami-cel, larotrectinib (tazemetostat was withdrawn in March 2026).
- Am I a candidate for Imatinib, Afamitresgene autoleucel, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Localised extremity/trunk soft-tissue sarcoma, low grade
- For my situation (localised extremity/trunk soft-tissue sarcoma, low grade), which of the standard options do you recommend and why?Guideline options include: Wide resection (limb-salvage) ± radiotherapy for margins or size >5 cm; observation thereafter.
Localised high-risk soft-tissue sarcoma (grade 3, >5 cm, deep)
- For my situation (localised high-risk soft-tissue sarcoma (grade 3, >5 cm, deep)), which of the standard options do you recommend and why?Guideline options include: Neoadjuvant anthracycline-ifosfamide × 3 (ISG-STS 1001) ± preoperative radiotherapy, then wide resection; regional hyperthermia with chemotherapy where available (EORTC 62961).
- 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 soft-tissue sarcoma, first line
- For my situation (advanced soft-tissue sarcoma, first line), which of the standard options do you recommend and why?Guideline options include: Doxorubicin 75 mg/m2 (single agent) or doxorubicin-ifosfamide for symptomatic/rapid disease (EORTC 62012: PFS but not OS benefit); histotype exceptions: trabectedin or gemcitabine-docetaxel for leiomyosarcoma, paclitaxel for angiosarcoma. Adding olaratumab to doxorubicin gave no survival benefit (ANNOUNCE).
- Am I a candidate for Doxorubicin, Ifosfamide, Trabectedin, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of ANNOUNCE apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Advanced soft-tissue sarcoma, later lines
- For my situation (advanced soft-tissue sarcoma, later lines), which of the standard options do you recommend and why?Guideline options include: Trabectedin (L-sarcomas), eribulin (liposarcoma), pazopanib (non-adipocytic), gemcitabine-docetaxel, dacarbazine; pembrolizumab for alveolar soft-part sarcoma or UPS; larotrectinib for NTRK fusion; afami-cel or lete-cel for MAGE-A4/NY-ESO-1+ synovial sarcoma and MRCLS.
- Am I a candidate for Trabectedin, Pazopanib, Pembrolizumab or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of IGNYTE-ESO apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
GIST, localised
- For my situation (gist, localised), which of the standard options do you recommend and why?Guideline options include: Resection; adjuvant imatinib 3 years for high risk (SSGXVIII), longer under study; none for PDGFRA D842V or SDH-deficient (imatinib-insensitive).
- Am I a candidate for Imatinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of SSGXVIII/AIO (adjuvant imatinib in GIST) apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
GIST, advanced
- For my situation (gist, advanced), which of the standard options do you recommend and why?Guideline options include: Imatinib 400 mg (800 mg for exon 9) → sunitinib → regorafenib → ripretinib (INVICTUS); avapritinib for PDGFRA D842V; ctDNA KIT genotyping to choose ripretinib vs sunitinib second line (INSIGHT); surgery for oligoprogression.
- Am I a candidate for Imatinib, Ripretinib, Avapritinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of INVICTUS and INSIGHT apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Osteosarcoma
- For my situation (osteosarcoma), which of the standard options do you recommend and why?Guideline options include: Neoadjuvant MAP (methotrexate, doxorubicin, cisplatin) → limb-salvage resection → adjuvant MAP; no benefit from adding ifosfamide-etoposide or interferon (EURAMOS-1); metastatic/relapsed: surgery of lung metastases, regorafenib/cabozantinib.
- Am I a candidate for Doxorubicin, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Ewing sarcoma
- For my situation (ewing sarcoma), which of the standard options do you recommend and why?Guideline options include: Interval-compressed VDC/IE (INT-0091, AEWS0031, Euro Ewing 2012) with surgery and/or radiotherapy for local control; high-dose busulfan-melphalan for selected high-risk (Euro-EWING 99 R2); relapse: irinotecan-temozolomide, cabozantinib, trials.
- 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 INT-0091 (Ewing sarcoma) and Euro Ewing 2012 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Desmoid tumour
- For my situation (desmoid tumour), which of the standard options do you recommend and why?Guideline options include: Active surveillance first (many regress); nirogacestat (DeFi) for progressing symptomatic disease; sorafenib alternative; surgery only for select sites; cryoablation for extra-abdominal tumours.
- Am I a candidate for Nirogacestat, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of DeFi apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Tenosynovial giant cell tumour
- For my situation (tenosynovial giant cell tumour), which of the standard options do you recommend and why?Guideline options include: Surgery for localised disease; vimseltinib (MOTION) or pexidartinib (REMS for hepatotoxicity) for diffuse disease not amenable to surgery.
- Am I a candidate for Vimseltinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of MOTION apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Any stage
- Are there clinical trials I could join, for example of FAP-2286 (177Lu / 68Ga), Carbon-ion therapy, HS-20093, Gotistobart?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 “Rarity limits trials”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Chemoresistance of most subtypes”. 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 to Investigate Efficacy & Safety of Intratumoral INT230-6 Compared to US Standard of Care in Adults With Soft Tissue Sarcomas (INVINCIBLE-3)Phase 3 · recruiting · NCT06263231A Multicenter, Randomized, Phase 3 Study to Assess the Efficacy and Safety of INtratumorally Administered INT230-6 (SHAO, VINblastine, CIsplatin) Compared With US Standard of Care in Adults With Locally Recurrent, InoperaBLE, or Metastatic Soft Tissue Sarcomas (INVINCIBLE-3)
- INSIGHTPhase 3 · active · NCT05734105Second-line GIST with KIT exon 11 + exon 17/18 mutations (ctDNA-selected): ripretinib vs sunitinib
- Ivosidenib in Participants With Locally Advanced or Metastatic Conventional Chondrosarcoma Untreated or Previously Treated With 1 Systemic Treatment RegimenPhase 3 · recruiting · NCT06127407A Phase 3, Multicenter, Double-blind, Randomized, Placebo-controlled Study of Ivosidenib in Participants ≥18 Years of Age With Locally Advanced or Metastatic Conventional Chondrosarcoma With an IDH1 Mutation, Untreated or Previously Treated With 1 Systemic Treatment Regimen
- Multi-center Study of TBI-1301 (INN: Mipetresgene Autoleucel; Mip-cel) in Patients With NY-ESO-1 Positive Synovial SarcomaPhase 3 · recruiting · NCT07174427Multi-center Study of TBI-1301 (INN: Mipetresgene Autoleucel; Mip-cel) in Patients With NY-ESO-1 Positive Synovial Sarcoma
- Phase III Trial of Anlotinib, Catequentinib in Advanced Alveolar Soft Part Sarcoma, Leiomyosarcoma, Synovial Sarcoma (APROMISS)Phase 3 · recruiting · NCT03016819A Phase III Study of AL3818 (Anlotinib, Catequentinib) Hydrochloride Monotherapy in Subjects With Metastatic or Advanced Alveolar Soft Part Sarcoma, Leiomyosarcoma and Synovial 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.
- Sarcomas (soft tissue, bone, GIST): the full pageSarcomas are dozens of rare cancers of bone and connective tissue. GIST was the first solid tumour cured-in-practice by a targeted pill; synovial sarcoma got the first TCR-T therapy.
- 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.
- Objective response rate (ORR): Objective response rate (ORR) is the percentage of patients whose tumours shrink by at least 30%.
- Sarcoma (tissue type): Cancer of the body's connective and supporting tissues: bone, muscle, fat, cartilage, blood vessels, fibrous tissue.
- Histotype-tailored therapy: Histotype-tailored therapy means choosing treatment by the specific sarcoma subtype (there are more than 70) rather than treating all sarcomas alike.
- Wide local excision: Cutting out a tumour together with a measured rim of normal-looking tissue around it, so that microscopic spread at the edge is removed too.
- Limb-salvage surgery: Removing a bone or soft-tissue sarcoma while keeping the arm or leg, rebuilding the bone with a metal endoprosthesis or a graft.
- 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.
- Progression-free survival (PFS): Progression-free survival (PFS) is how long patients live without their cancer growing.
- HLA-A*02:01 restriction: Some T-cell-receptor drugs only work in people with a particular immune 'tissue type'.
- Dose-dense and metronomic chemotherapy: Two opposite ways of rescheduling the same drugs: dose-dense gives standard doses more often (every two weeks instead of three, supported by growth factors) to deny the tumour recovery time; metronomic gives small doses continuously to attack tumour blood vessels with little toxicity.
- Second cancers after radiotherapy: Radiotherapy can itself cause a new cancer in the treated area, typically ten to thirty years later.
Every term links to the glossary.