Synovial sarcoma
Synovial sarcoma is a young person's sarcoma driven by a single fusion gene, SS18-SSX, that scrambles how genes are switched on. It is treated with surgery, radiotherapy and ifosfamide-based chemotherapy, and in 2024 it became the first solid tumour with an approved engineered T-cell receptor therapy.
Overview
Synovial sarcoma has nothing to do with the synovium; it is defined by the SS18-SSX fusion, which hijacks the BAF chromatin remodelling complex. It presents as a slow-growing deep mass near the knee, ankle or other joints in people aged 15 to 40, and is graded high by default. Treatment is wide resection with radiotherapy, and chemotherapy with ifosfamide and doxorubicin is used more readily than in other sarcomas because responses are frequent, including in the neoadjuvant setting; pazopanib and trabectedin have activity in advanced disease. Most tumours express the cancer-testis antigens MAGE-A4 and NY-ESO-1, and afamitresgene autoleucel, a MAGE-A4-directed T-cell receptor therapy, was approved in the United States in 2024 for advanced disease in HLA-A*02 patients, with letetresgene autoleucel against NY-ESO-1 following in trials.
State of the art
- Afamitresgene autoleucel is the first T-cell receptor therapy approved for any solid tumour, with responses in about 40 percent of heavily pretreated patients.
- Chemosensitivity sets synovial sarcoma apart and justifies perioperative chemotherapy in high-risk cases.
- The single fusion driver makes it a model for epigenetic drugs such as BRD9 degraders.
Anatomy and lymph node drainage
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.
- Metaphysis, near the growth plate (osteosarcoma)
- Shaft (Ewing sarcoma)Poorly differentiated (round cell) synovial sarcoma
- Deep soft tissue compartmentMonophasic synovial sarcoma · Biphasic synovial sarcoma · Poorly differentiated (round cell) synovial sarcoma
- Skeletal muscle (rhabdomyosarcoma)
- Neurovascular bundle (limb salvage decision)
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Leiomyosarcoma, Liposarcoma, Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma, Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma)
Five to ten percent of soft-tissue sarcomas, typically in adolescents and young adults near the joints of the limbs; about half eventually metastasise, often to the lungs, and it is one of the few sarcomas where chemotherapy clearly helps.
- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
Wide resection with pre- or postoperative radiotherapy; neoadjuvant or adjuvant ifosfamide-doxorubicin for large high-risk tumours.
Ifosfamide-based chemotherapy, doxorubicin; pazopanib or trabectedin later.
Afamitresgene autoleucel after chemotherapy; NY-ESO-1 TCR therapy in trials.
Subtypes & biomarkers
top- Monophasic synovial sarcoma
- Biphasic synovial sarcoma
- Poorly differentiated (round cell) synovial sarcoma
- SS18-SSX fusion (FISH or RNA sequencing; SS18-SSX antibody)
- MAGE-A4 and NY-ESO-1 expression with HLA-A*02 typing (TCR therapy eligibility)
- Size over 5 cm and poorly differentiated component (prognosis)
How often this target appears
- 1994SS18-SSX fusion identified
- 2012Pazopanib approved for soft-tissue sarcoma including synovial sarcoma
- 2024Afamitresgene autoleucel approved: first TCR therapy for a solid tumour
What is in development for Synovial sarcoma, drawn from the whole corpus: 0 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Nothing recorded in development for this cancer yet.
Open problems and what is being done
TCR therapy needs a matching HLA type and antigen, excluding most patients.
Late lung metastases years after treatment.
and how the field plans to fix it →What is being done about thisAdvanced and metastatic diseaseAvailable now- IMRT / IGRT (modern external beam)Standard of care
- Bone-modifying agents (bisphosphonates, denosumab)Standard of care
- DordaviproneApproved
- HIPEC / PIPAC (intraperitoneal chemotherapy)Established
- Laser interstitial thermal therapy (LITT)Established
- Liquid biopsy (ctDNA)Standard of care
In trials- ACTIONRecruiting
- CAR-T for glioma (IL13Rα2, GD2, EGFRvIII, multi-target)Phase 1
- CIRCULATE-Japan (GALAXY / VEGA / ALTAIR)Active
- DESTINY-Breast12Positive
- DYNAMICPositive
- EF-14Positive
Ideas and roadmaps- A billion-dollar prize for the first durable cure of a lethal metastatic cancer
- A blood test for the pre-metastatic niche
- A global rapid tissue donation network for metastatic disease
- A national rapid research autopsy network for end-stage cancer
- A regulatory endpoint for drugs that block spread, not tumours
- A ring-fenced metastasis programme with metastasis-specific endpoints
Background: Blood-brain barrier (BBB), Circulating tumour DNA (ctDNA), EGFRvIII, Epithelial-mesenchymal transition & drug efflux, H3 K27M (diffuse midline glioma). Also on OnCo: Atlas of advanced disease · Invasion and metastasis.
Fertility and growth in adolescent patients receiving chemotherapy.
Trials
topTrials recruiting now
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Expert centres
topExpert centres
- Aarhus University HospitalAarhus, DKvia IMRT / IGRT (modern external beam)
- American Society for Radiation OncologyArlington, VA, USvia IMRT / IGRT (modern external beam)
- Centre Antoine LacassagneNice, FRvia IMRT / IGRT (modern external beam)
- Centre Oscar LambretLille, FRvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Comprehensive Cancer Center Freiburg (CCCF)Freiburg im Breisgau, DEvia IMRT / IGRT (modern external beam)
- Comprehensive Cancer Center Tübingen-StuttgartTübingen, DEvia Afamitresgene autoleucel
- European Society for Radiotherapy and OncologyBrussels, BEvia IMRT / IGRT (modern external beam)
- Geneva University Hospitals (HUG)Geneva, CHvia IMRT / IGRT (modern external beam)
- Groote Schuur Hospital / University of Cape TownCape Town, ZAvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Hacettepe University Cancer InstituteAnkara, TRvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Ho Chi Minh City Oncology HospitalHo Chi Minh City, VNvia IMRT / IGRT (modern external beam)
- Hokkaido University HospitalSapporo, JPvia IMRT / IGRT (modern external beam)
- Hunan Cancer HospitalChangsha, CNvia IMRT / IGRT (modern external beam)
- Institut BergoniéBordeaux, FRvia IMRT / IGRT (modern external beam)
- Institut National d'Oncologie, RabatRabat, MAvia IMRT / IGRT (modern external beam)
- Institut Salah AzaïezTunis, TNvia IMRT / IGRT (modern external beam)
- Institute of Oncology LjubljanaLjubljana, SIvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- International Extranodal Lymphoma Study GroupBellinzona, CHvia IMRT / IGRT (modern external beam)
- IRCCS Humanitas Research HospitalRozzano (Milan), ITvia IMRT / IGRT (modern external beam)
- Istanbul University Institute of OncologyIstanbul, TRvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Juravinski Cancer Centre / Escarpment Cancer Research InstituteHamilton, ON, CAvia IMRT / IGRT (modern external beam)
- Kenyatta National HospitalNairobi, KEvia IMRT / IGRT (modern external beam)
- Korle Bu Teaching HospitalAccra, GHvia IMRT / IGRT (modern external beam)
- Lagos University Teaching HospitalLagos, NGvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- National Institute of Oncology, HungaryBudapest, HUvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Ocean Road Cancer InstituteDar es Salaam, TZvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Rajiv Gandhi Cancer Institute and Research CentreNew Delhi, INvia IMRT / IGRT (modern external beam)
- Rambam Health Care CampusHaifa, ILvia IMRT / IGRT (modern external beam)
- Rigshospitalet, Copenhagen University HospitalCopenhagen, DKvia IMRT / IGRT (modern external beam)
- Royal Adelaide HospitalAdelaide, AUvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Siriraj Hospital, Mahidol UniversityBangkok, THvia IMRT / IGRT (modern external beam)
- Tata Medical Center, KolkataKolkata, INvia IMRT / IGRT (modern external beam)
- Tawam HospitalAl Ain, AEvia IMRT / IGRT (modern external beam)
- Tel Aviv Sourasky Medical CenterTel Aviv, ILvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- TROG Cancer ResearchNewcastle, NSW, AUvia IMRT / IGRT (modern external beam)
- UMC Utrecht Cancer CenterUtrecht, NLvia IMRT / IGRT (modern external beam)
- University of Malaya Medical CentreKuala Lumpur, MYvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Velindre Cancer CentreCardiff, GBvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Zhejiang Cancer HospitalHangzhou, CNvia IMRT / IGRT (modern external beam)
Questions to ask
topQuestions to ask your oncologist about Synovial sarcoma
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example SS18-SSX fusion, MAGE-A4 and NY-ESO-1 expression with HLA-A*02 typing, Size over 5 cm and poorly differentiated component), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Monophasic synovial sarcoma, Biphasic synovial sarcoma, Poorly differentiatedsynovial sarcoma.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Localised
- For my situation (localised), which of the standard options do you recommend and why?Why: Guideline options include: Wide resection with pre- or postoperative radiotherapy; neoadjuvant or adjuvant ifosfamide-doxorubicin for large high-risk tumours.
- Am I a candidate for Ifosfamide, Doxorubicin, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced, first line
- For my situation (advanced, first line), which of the standard options do you recommend and why?Why: Guideline options include: Ifosfamide-based chemotherapy, doxorubicin; pazopanib or trabectedin later.
- Am I a candidate for Ifosfamide, Doxorubicin, Pazopanib or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced, HLA-A*02 and MAGE-A4-positive
- For my situation (advanced, hla-a*02 and mage-a4-positive), which of the standard options do you recommend and why?Why: Guideline options include: Afamitresgene autoleucel after chemotherapy; NY-ESO-1 TCR therapy in trials.
- Am I a candidate for Afamitresgene autoleucel, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: 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?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “TCR therapy needs a matching HLA type and antigen, excluding most patients”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Late lung metastases years after treatment”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
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Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
6targets
3drugs
5companies
3Latest papers
topQuery for this cancer: (TITLE:"Synovial sarcoma" OR ABSTRACT:"Synovial sarcoma" OR TITLE:"SS18-SSX sarcoma" OR ABSTRACT:"SS18-SSX sarcoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Synovial sarcoma, not a curated reading list.
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