OnCo

Sarcomas (soft tissue, bone, GIST): lines of therapy

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12 standard-of-care settings across 4 lines and 2 biomarker subgroups. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.

LineAll comersRisk group
Early / localised31
Advanced, first line3·
Third line and beyond1·
Other settings4·

Early / localised

SubgroupSettingApproachProducts and trialsEvidence
All comersLocalised STSWide excision + radiation; neoadjuvant chemotherapy for high-risk.91
All comersLocalised extremity/trunk soft-tissue sarcoma, low gradeWide resection (limb-salvage) ± radiotherapy for margins or size >5 cm; observation thereafter.91
All comersGIST, localisedResection; adjuvant imatinib 3 years for high risk (SSGXVIII), longer under study; none for PDGFRA D842V or SDH-deficient (imatinib-insensitive).78
Risk groupLocalised 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).91

Advanced, first line

SubgroupSettingApproachProducts and trialsEvidence
All comersAdvancedDoxorubicin ± ifosfamide; subtype-directed: imatinib, afami-cel, larotrectinib (tazemetostat was withdrawn in March 2026).78
All comersAdvanced soft-tissue sarcoma, first lineDoxorubicin 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.79
All comersGIST, advancedImatinib 400 mg (800 mg for exon 9) → sunitinibregorafenibripretinib (INVICTUS); avapritinib for PDGFRA D842V; ctDNA KIT genotyping to choose ripretinib vs sunitinib second line (INSIGHT); surgery for oligoprogression.78

Third line and beyond

SubgroupSettingApproachProducts and trialsEvidence
All comersAdvanced soft-tissue sarcoma, later linesTrabectedin (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.98

Other settings

SubgroupSettingApproachProducts and trialsEvidence
All comersOsteosarcomaNeoadjuvant 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.79
All comersEwing sarcomaInterval-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.79
All comersDesmoid tumourActive surveillance first (many regress); nirogacestat (DeFi) for progressing symptomatic disease; sorafenib alternative; surgery only for select sites; cryoablation for extra-abdominal tumours.77
All comersTenosynovial giant cell tumourSurgery for localised disease; vimseltinib (MOTION) or pexidartinib (REMS for hepatotoxicity) for diffuse disease not amenable to surgery.70

Sequence and caution pairings

Regimens in the library

Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.