Liposarcoma
Liposarcoma is a cancer of fat cells that comes in four different forms: two driven by extra copies of the MDM2 gene, one by a fusion gene that makes it unusually sensitive to radiotherapy and trabectedin, and one that behaves like other aggressive sarcomas. Surgery is the mainstay and MDM2 inhibitors are the most promising drugs in trials.
Overview
Well-differentiated and dedifferentiated liposarcoma share amplification of MDM2 and CDK4 on chromosome 12 and arise mostly in the retroperitoneum and thigh; the well-differentiated form does not metastasise but recurs locally, and the dedifferentiated form is aggressive. Myxoid liposarcoma carries the FUS-DDIT3 fusion, occurs in younger adults, spreads to unusual soft-tissue and bone sites and is strikingly sensitive to radiotherapy and to trabectedin. Pleomorphic liposarcoma is genomically complex and treated like other high-grade sarcomas. Surgery is the mainstay, with radiotherapy for limb tumours and, for retroperitoneal disease, the STRASS trial failed to show a benefit of preoperative radiotherapy overall. Eribulin improved survival in advanced liposarcoma, trabectedin is effective in myxoid disease, and MDM2 inhibitors such as brigimadlin and CDK4/6 inhibitors are in late trials for the MDM2-amplified forms.
State of the art
- MDM2 amplification is both the diagnostic test and the therapeutic target, with brigimadlin in a phase 3 trial against doxorubicin.
- Myxoid liposarcoma's radiosensitivity allows reduced-dose preoperative radiotherapy.
Show survival figures (1)
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
- Eribulin was the first drug to lengthen survival specifically in liposarcoma.
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)Myxoid and round cell liposarcoma (FUS-DDIT3)
- Deep soft tissue compartmentWell-differentiated liposarcoma (atypical lipomatous tumour; MDM2-amplified) · Dedifferentiated liposarcoma (MDM2-amplified, aggressive) · Myxoid and round cell liposarcoma (FUS-DDIT3) · Pleomorphic liposarcoma
- Skeletal muscle (rhabdomyosarcoma)Pleomorphic liposarcoma
- 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, Synovial sarcoma, 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)
The commonest soft-tissue sarcoma of adults, about a fifth of the total; outcomes range from near-benign well-differentiated tumours of the limbs to dedifferentiated retroperitoneal tumours that recur relentlessly and kill.
- 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.
Marginal excision; no radiotherapy or systemic therapy; surveillance for local recurrence.
Complete en bloc resection in a sarcoma centre; preoperative radiotherapy not routine after STRASS; repeat surgery for recurrence.
Surgery with preoperative radiotherapy (highly radiosensitive); trabectedin for advanced disease.
Doxorubicin-based chemotherapy, eribulin (overall survival benefit in liposarcoma), trabectedin; MDM2 and CDK4 inhibitors in trials for amplified tumours.
Subtypes & biomarkers
top- Well-differentiated liposarcoma (atypical lipomatous tumour; MDM2-amplified)
- Dedifferentiated liposarcoma (MDM2-amplified, aggressive)
- Myxoid and round cell liposarcoma (FUS-DDIT3)
- Pleomorphic liposarcoma
- MDM2 and CDK4 amplification (FISH; separates from lipoma)
- FUS-DDIT3 fusion (myxoid)
- Round cell component (myxoid prognosis)
- Grade and site (retroperitoneal versus limb)
How often this target appears
- 1962Enzinger and Winslow classify liposarcoma
- 1993MDM2 amplification found in well-differentiated and dedifferentiated liposarcoma
- 2016Eribulin approved for liposarcoma after a survival benefit
- 2020STRASS: preoperative radiotherapy does not improve retroperitoneal sarcoma outcomes overall
What is in development for Liposarcoma, 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
Retroperitoneal dedifferentiated disease recurs in most patients despite complete surgery.
MDM2 inhibitors cause low platelets that limit dosing.
No systemic therapy is proven in the adjuvant setting.
Trials
topTrials recruiting now
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Expert centres
topExpert centres
- via Sarcomas (soft tissue, bone, GIST)
- via Sarcomas (soft tissue, bone, GIST)
- via Sarcomas (soft tissue, bone, GIST)
- via Sarcomas (soft tissue, bone, GIST)
- via Sarcomas (soft tissue, bone, GIST)
- Centre Oscar LambretLille, FRvia Sarcomas (soft tissue, bone, GIST), IMRT / IGRT (modern external beam)
- via Sarcomas (soft tissue, bone, GIST), IMRT / IGRT (modern external beam)
- Institut BergoniéBordeaux, FRvia Sarcomas (soft tissue, bone, GIST), IMRT / IGRT (modern external beam)
- via Sarcomas (soft tissue, bone, GIST), IMRT / IGRT (modern external beam)
- via Sarcomas (soft tissue, bone, GIST), IMRT / IGRT (modern external beam)
- Tel Aviv Sourasky Medical CenterTel Aviv, ILvia Sarcomas (soft tissue, bone, GIST), IMRT / IGRT (modern external beam)
- A.C. Camargo Cancer CenterSão Paulo, BRvia Sarcomas (soft tissue, bone, GIST)
- 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 Léon BérardLyon, FRvia Sarcomas (soft tissue, bone, GIST)
- via IMRT / IGRT (modern external beam)
- Children's Cancer and Leukaemia GroupLeicester, GBvia Sarcomas (soft tissue, bone, GIST)
- Children's Oncology Group (COG)Monrovia, CA, USvia Sarcomas (soft tissue, bone, GIST)
- Chris O'Brien LifehouseSydney, AUvia Sarcomas (soft tissue, bone, GIST)
- Comprehensive Cancer Center Freiburg (CCCF)Freiburg im Breisgau, DEvia IMRT / IGRT (modern external beam)
- EORTCBrussels, BEvia Sarcomas (soft tissue, bone, GIST)
- Erasmus MC Cancer InstituteRotterdam, NLvia Sarcomas (soft tissue, bone, GIST)
- European Society for Radiotherapy and OncologyBrussels, BEvia IMRT / IGRT (modern external beam)
- via Sarcomas (soft tissue, bone, GIST)
- 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)
- Gunma University Heavy Ion Medical CenterMaebashi, JPvia Sarcomas (soft tissue, bone, GIST)
- 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 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)
- International Extranodal Lymphoma Study GroupBellinzona, CHvia IMRT / IGRT (modern external beam)
- IRCCS Humanitas Research HospitalRozzano (Milan), ITvia IMRT / IGRT (modern external beam)
- via Sarcomas (soft tissue, bone, GIST)
- 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)
- Leiden University Medical CenterLeiden, NLvia Sarcomas (soft tissue, bone, GIST)
- 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 Sarcomas (soft tissue, bone, GIST)
- via IMRT / IGRT (modern external beam)
- Peking University Cancer HospitalBeijing, CNvia Sarcomas (soft tissue, bone, GIST)
- via Sarcomas (soft tissue, bone, GIST)
- via Sarcomas (soft tissue, bone, GIST)
- 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)
- Sarcoma Oncology CenterSanta Monica, USvia Sarcomas (soft tissue, bone, GIST)
- via IMRT / IGRT (modern external beam)
- via Sarcomas (soft tissue, bone, GIST)
- Siriraj Hospital, Mahidol UniversityBangkok, THvia IMRT / IGRT (modern external beam)
- Society of Surgical OncologyRosemont, IL, USvia Sarcomas (soft tissue, bone, GIST)
- via Sarcomas (soft tissue, bone, GIST)
- Tata Medical Center, KolkataKolkata, INvia IMRT / IGRT (modern external beam)
- Tawam HospitalAl Ain, AEvia IMRT / IGRT (modern external beam)
- The Ohio State University Comprehensive Cancer Center, James Cancer Hospital and Solove Research InstituteColumbus, OH, USNCI comprehensivevia Sarcomas (soft tissue, bone, GIST)
- 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)
- via Sarcomas (soft tissue, bone, GIST)
- Zhejiang Cancer HospitalHangzhou, CNvia IMRT / IGRT (modern external beam)
Questions to ask
topQuestions to ask your oncologist about Liposarcoma
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 MDM2 and CDK4 amplification, FUS-DDIT3 fusion, Round cell component, Grade and site), 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 Well-differentiated liposarcoma, Dedifferentiated liposarcoma, Myxoid and round cell liposarcoma.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Well-differentiated, limb
- For my situation (well-differentiated, limb), which of the standard options do you recommend and why?Why: Guideline options include: Marginal excision; no radiotherapy or systemic therapy; surveillance for local recurrence.
Retroperitoneal well- and dedifferentiated
- For my situation (retroperitoneal well- and dedifferentiated), which of the standard options do you recommend and why?Why: Guideline options include: Complete en bloc resection in a sarcoma centre; preoperative radiotherapy not routine after STRASS; repeat surgery for recurrence.
Myxoid liposarcoma
- For my situation (myxoid liposarcoma), which of the standard options do you recommend and why?Why: Guideline options include: Surgery with preoperative radiotherapy (highly radiosensitive); trabectedin for advanced disease.
- Am I a candidate for Trabectedin, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced disease
- For my situation (advanced disease), which of the standard options do you recommend and why?Why: Guideline options include: Doxorubicin-based chemotherapy, eribulin (overall survival benefit in liposarcoma), trabectedin; MDM2 and CDK4 inhibitors in trials for amplified tumours.
- Am I a candidate for Doxorubicin, Eribulin, Trabectedin, 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 “Retroperitoneal dedifferentiated disease recurs in most patients despite complete surgery”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “MDM2 inhibitors cause low platelets that limit dosing”. 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
3targets
1drugs
3companies
2Latest papers
topQuery for this cancer: (TITLE:"Liposarcoma" OR ABSTRACT:"Liposarcoma" OR TITLE:"Well-differentiated liposarcoma atypical lipomatous tumour" OR ABSTRACT:"Well-differentiated liposarcoma atypical lipomatous tumour" OR TITLE:"Dedifferentiated liposarcoma" OR ABSTRACT:"Dedifferentiated liposarcoma" OR TITLE:"Myxoid liposarcoma" OR ABSTRACT:"Myxoid liposarcoma" OR TITLE:"Pleomorphic liposarcoma" OR ABSTRACT:"Pleomorphic liposarcoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Liposarcoma, not a curated reading list.
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