• 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.

Biomarker results to ask for: Histologic subtype, KIT/PDGFRA (GIST), MAGE-A4 + HLA-A*02, NTRK fusions, INI1 loss, CDK4/MDM2 amplification (liposarcoma), Histologic subtype and FNCLCC grade, KIT/PDGFRA mutation and secondary mutations (tissue or ctDNA) in GIST, SS18-SSX, EWSR1-FLI1, FUS-DDIT3, ASPSCR1-TFE3 fusions, MAGE-A4 or NY-ESO-1 expression + HLA-A*02 for TCR-T, INI1 (SMARCB1) loss, MDM2/CDK4 amplification (liposarcoma), CTNNB1/APC mutation (desmoid), CSF1 translocation (TGCT), Sarculator nomogram risk.

Scans and tests linked to this cancer: Comprehensive genomic profiling, Liquid biopsy (ctDNA), Intraoperative MRI and CT, Strain echocardiography (global longitudinal strain), Troponin and natriuretic peptide monitoring during cancer treatment, FAPI PET.

Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.

• Localised STS: Wide excision + radiation; neoadjuvant chemotherapy for high-risk. (IMRT / IGRT (modern external beam), Proton therapy, Hyperthermia)
• Localised extremity/trunk soft-tissue sarcoma, low grade: Wide resection (limb-salvage) ± radiotherapy for margins or size >5 cm; observation thereafter. (Limb-salvage surgery and endoprosthetic reconstruction, IMRT / IGRT (modern external beam), FNCLCC grade (soft-tissue sarcoma))
• GIST, localised: Resection; adjuvant imatinib 3 years for high risk (SSGXVIII), longer under study; none for PDGFRA D842V or SDH-deficient (imatinib-insensitive). (Imatinib, SSGXVIII/AIO (adjuvant imatinib in GIST))
• Localised high-risk soft-tissue sarcoma (grade 3, >5 cm, deep): Neoadjuvant anthracycline-ifosfamide × 3 (ISG-STS 1001) ± preoperative radiotherapy, then wide resection; regional hyperthermia with chemotherapy where available (EORTC 62961). (ISG-STS 1001, Doxorubicin, Ifosfamide, IMRT / IGRT (modern external beam), Hyperthermia, Limb-salvage surgery and endoprosthetic reconstruction)
• Advanced: Doxorubicin ± ifosfamide; subtype-directed: imatinib, afami-cel, larotrectinib (tazemetostat was withdrawn in March 2026). (Imatinib, Afamitresgene autoleucel)
• Advanced soft-tissue sarcoma, first line: 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). (Doxorubicin, Ifosfamide, Trabectedin, ANNOUNCE, Histotype-tailored therapy)
• GIST, advanced: 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. (Imatinib, Ripretinib, INVICTUS, INSIGHT, Avapritinib, ctDNA KIT genotyping → TKI selection in GIST)
• Osteosarcoma: 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. (Doxorubicin, Limb-salvage surgery and endoprosthetic reconstruction, Children's Oncology Group (COG))
• Ewing sarcoma: 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. (INT-0091 (Ewing sarcoma), Euro Ewing 2012, Doxorubicin, Ifosfamide, Proton therapy)
• Desmoid tumour: Active surveillance first (many regress); nirogacestat (DeFi) for progressing symptomatic disease; sorafenib alternative; surgery only for select sites; cryoablation for extra-abdominal tumours. (Nirogacestat, DeFi, Thermal ablation (RFA, microwave, cryo), Intermittent or stop-and-restart nirogacestat in desmoid tumours)
• Tenosynovial giant cell tumour: Surgery for localised disease; vimseltinib (MOTION) or pexidartinib (REMS for hepatotoxicity) for diffuse disease not amenable to surgery. (Vimseltinib, MOTION)
• Advanced soft-tissue sarcoma, later lines: 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. (Trabectedin, Pazopanib, Pembrolizumab, Afamitresgene autoleucel, Letetresgene autoleucel, IGNYTE-ESO)

From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.