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10 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
Sarcomas are cancers of connective tissue: more than 70 subtypes of soft-tissue sarcoma (liposarcoma, leiomyosarcoma, undifferentiated pleomorphic sarcoma, synovial sarcoma, angiosarcoma and others), bone sarcomas (osteosarcoma, Ewing sarcoma, chondrosarcoma), gastrointestinal stromal tumour (GIST), and locally aggressive but non-metastasising tumours such as desmoid fibromatosis and tenosynovial giant cell tumour. Together they are about 1% of adult and 15% of childhood cancers. Because each subtype is rare, expertise concentrates in reference centres, and treatment is increasingly by histotype and genotype: FNCLCC grade, size and depth define risk in soft-tissue sarcoma; KIT/PDGFRA mutations define GIST therapy; fusion genes (SS18-SSX, EWSR1-FLI1, FUS-DDIT3) define entities and, increasingly, targets.
Surgery with negative margins remains the curative act, with limb-salvage now standard and radiotherapy improving local control in soft-tissue sarcoma. Neoadjuvant anthracycline-ifosfamide benefits high-risk localised soft-tissue sarcoma (ISG-STS 1001), and multi-agent chemotherapy cures the majority of localised osteosarcoma (MAP) and Ewing sarcoma (VDC/IE, INT-0091 and Euro Ewing 2012). Advanced soft-tissue sarcoma still depends on doxorubicin with a median survival around 18-20 months; olaratumab's failure (ANNOUNCE) showed how hard that bar is to move. Subtype-specific drugs then fill in: trabectedin and eribulin for L-sarcomas, pazopanib for non-adipocytic sarcomas, and, in GIST, the sequence imatinib (3 years adjuvant, SSGXVIII), sunitinib, regorafenib and ripretinib (INVICTUS), with avapritinib for PDGFRA D842V and ctDNA-genotype-directed selection (INSIGHT) arriving. Desmoid tumours gained their first drug in nirogacestat (DeFi, 2023) and tenosynovial giant cell tumour its second in vimseltinib (MOTION, February 2025); tazemetostat for epithelioid sarcoma was withdrawn worldwide in March 2026 over secondary blood cancers.
| Setting | Approach | Guideline |
|---|---|---|
| Localised STS | Wide excision + radiation; neoadjuvant chemotherapy for high-risk. | not mapped |
| Advanced | Doxorubicin ± ifosfamide; subtype-directed: imatinib, afami-cel, larotrectinib (tazemetostat was withdrawn in March 2026). | not mapped |
| Localised extremity/trunk soft-tissue sarcoma, low grade | Wide resection (limb-salvage) ± radiotherapy for margins or size >5 cm; observation thereafter. | NCCN Category 1 (surgery ± RT) |
| 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). | NCCN Category 2A (neoadjuvant chemotherapy for high-risk), ESMO-MCBS A (ISG-STS 1001) |
| 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). | NCCN Category 1 (anthracycline-based) |
| 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. | NCCN Category 2A |
| GIST, localised | Resection; adjuvant imatinib 3 years for high risk (SSGXVIII), longer under study; none for PDGFRA D842V or SDH-deficient (imatinib-insensitive). | NCCN Category 1 (imatinib 3 years, high-risk), ESMO-MCBS A |
| 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. | NCCN Category 1 sequence |