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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giant cell tumour its second in vimseltinib (MOTION, February 2025); tazemetostat for epithelioid sarcoma was withdrawn worldwide in March 2026 over secondary blood cancers.
Sarcoma is also where solid-tumour T-cell engineering first succeeded: afamitresgene autoleucel (MAGE-A4 TCR-T) was approved in 2024 and fully approved with extension to adolescents in June 2026, and letetresgene autoleucel (NY-ESO-1) has a BLA due by end-2026. Checkpoint inhibitors help a minority (alveolar soft-part sarcoma, UPS, angiosarcoma); most sarcomas are immunologically cold. The unsolved problems are metastatic osteosarcoma and Ewing sarcoma (survival unchanged in 30 years), the HLA restriction of TCR therapies, chemoresistance of most adult subtypes, and trial feasibility in diseases with a few hundred cases a year.
Sarcomas make up ~1% of adult cancers (~13,500 soft-tissue and ~3,900 bone sarcomas in the US per year) and ~15% of paediatric cancers, across >70 histologic subtypes.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
Bone sarcomas favour the fast-growing ends of long bones (osteosarcoma) or the shaft (Ewing), soft tissue sarcomas the deep muscle compartments; spread is through the blood to the lungs, rarely via lymph nodes.
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Adamantinoma of bone, Dedifferentiated chordoma, Poorly differentiated chordoma (SMARCB1-deficient), Desmoplastic small round cell tumour, Leiomyosarcoma, Liposarcoma, Synovial sarcoma, Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma, Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), Chondrosarcoma, Angiosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Myxofibrosarcoma, Alveolar soft part sarcoma, Perivascular epithelioid cell tumour (PEComa), Epithelioid haemangioendothelioma, Malignant peripheral nerve sheath tumour (MPNST), Retroperitoneal sarcoma, Soft tissue sarcoma of the extremity (localised and advanced)
Most people reading this do not have advanced disease. The map describes what can happen over the whole course of the illness, across autopsy and registry series; today's staging scans find spread earlier, and each site has treatments, from focused radiotherapy for a few spots to drugs that reach the brain.
What helpsLung metastasectomy is standard and can cure; doxorubicin-based chemotherapy, imatinib and its successors for GIST, afami-cel TCR-T for synovial sarcoma.
Also on OnCo: Atlas of advanced disease · How cancer spreads: the metastasis stages.
Sarcomas spread through the blood almost exclusively to the lungs; lung metastasectomy is standard.
Except epithelioid, synovial, rhabdomyosarcoma and clear cell subtypes.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Wide excision + radiation; neoadjuvant chemotherapy for high-risk.
Doxorubicin ± ifosfamide; subtype-directed: imatinib, afami-cel, larotrectinib (tazemetostat was withdrawn in March 2026).
Wide resection (limb-salvage) ± radiotherapy for margins or size >5 cm; observation thereafter.
Neoadjuvant anthracycline-ifosfamide × 3 (ISG-STS 1001) ± preoperative radiotherapy, then wide resection; regional hyperthermia with chemotherapy where available (EORTC 62961).
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).
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.
Resection; adjuvant imatinib 3 years for high risk (SSGXVIII), longer under study; none for PDGFRA D842V or SDH-deficient (imatinib-insensitive).
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.
Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask.
Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.