Leiomyosarcoma
Leiomyosarcoma is a cancer of smooth muscle, most often in the womb, the abdomen behind the bowel or the wall of a large vein. Surgery is the only cure; for spread disease, doxorubicin-based chemotherapy, trabectedin and gemcitabine-docetaxel are the standards, with no targeted drug yet.
Overview
Leiomyosarcoma arises from smooth muscle and is genomically complex, with TP53, RB1 and ATRX loss and no recurrent fusion. Uterine leiomyosarcoma is usually found unexpectedly after surgery for presumed fibroids, which is why morcellation of unsuspected tumours is avoided; retroperitoneal and vascular tumours present late. Complete surgical resection, with radiotherapy for limb tumours, is the only curative treatment and adjuvant chemotherapy has not proven benefit. Metastatic disease is treated with doxorubicin, alone or with trabectedin (LMS-04) or dacarbazine, gemcitabine-docetaxel, trabectedin and pazopanib; responses are modest and median survival is about two years. Immunotherapy has little activity, and trials target the disease's DNA repair and cell-cycle vulnerabilities.
State of the art
- Genomic profiling has defined the disease but not yet yielded a targeted drug.
Show survival figures (2)
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.
- LMS-04 gave the first randomised progression-free survival gain in a decade by combining doxorubicin with trabectedin.
- Lung metastasectomy in selected patients produces long survival that chemotherapy alone does not.
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)
- Deep soft tissue compartmentUterine leiomyosarcoma · Retroperitoneal and intra-abdominal leiomyosarcoma · Vascular leiomyosarcoma (inferior vena cava, large veins) · Limb and cutaneous leiomyosarcoma (better outlook)
- 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: Liposarcoma, 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)
One of the two commonest soft-tissue sarcomas in adults, arising in the uterus, the retroperitoneum, large veins and limbs; about half of patients with deep tumours eventually develop metastases, most often to the lungs.
- 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 surgical resection; radiotherapy for limb and trunk tumours; no morcellation of uterine masses that could be sarcoma; adjuvant chemotherapy not standard.
Doxorubicin plus trabectedin (LMS-04) or doxorubicin alone; gemcitabine-docetaxel as an alternative, especially in uterine disease.
Trabectedin, pazopanib, dacarbazine-based regimens, aromatase inhibitors in hormone receptor-positive low-grade uterine disease; metastasectomy for limited lung spread.
Subtypes & biomarkers
top- Uterine leiomyosarcoma
- Retroperitoneal and intra-abdominal leiomyosarcoma
- Vascular leiomyosarcoma (inferior vena cava, large veins)
- Limb and cutaneous leiomyosarcoma (better outlook)
- TP53, RB1 and ATRX alterations
- Grade (FNCLCC) and size
- Smooth muscle markers (desmin, h-caldesmon, SMA)
- Oestrogen and progesterone receptor in uterine tumours (hormone therapy in low-grade disease)
How often this target appears
- 1994Doxorubicin-ifosfamide becomes the reference first-line regimen for soft-tissue sarcoma
- 2007Trabectedin approved in Europe for soft-tissue sarcoma
- 2014Morcellation warnings after unsuspected uterine leiomyosarcoma spread
- 2022LMS-04: doxorubicin plus trabectedin improves progression-free survival
What is in development for Leiomyosarcoma, 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
No targeted or immune therapy works in most patients.
and how the field plans to fix it →What is being done about thisCancers without a drug targetAvailable now- AdagrasibApproved
- CRISPR functional genomicsEstablished
- DaraxonrasibApproved
- KRAS & RAS inhibitorsApproved
- PROTACs & molecular glues (targeted protein degradation)Approved
- RevumenibApproved
In trials- AI-driven drug & target discoveryPhase 2
- CodeBreaK 200Positive
- ELI-002 7PPhase 2
- ElironrasibPhase 2
- KRYSTAL-12Positive
- MRTX1133Phase 1
Ideas and roadmaps- A drug screen that only rewards killing sleeping cancer cells
- A guaranteed purchase prize for the first drug against a named hard target
- A precompetitive consortium for the twenty hardest cancer targets
- A synthetic lethality map for every cancer driver in every tissue context
- A target de-risking index that counts failures as well as successes
- AI-designed proteins that grip the floppy parts of cancer drivers
Background: p53 / RB / cell-cycle checkpoint, RAS / RAF / MEK / ERK (MAPK). Also on OnCo: Targets · KRAS roadmap.
Uterine tumours are still discovered by accident after fibroid surgery.
Adjuvant chemotherapy remains unproven.
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)
- European Society for Radiotherapy and OncologyBrussels, BEvia IMRT / IGRT (modern external beam)
- Geneva University Hospitals (HUG)Geneva, CHvia IMRT / IGRT (modern external beam)
- via Docetaxel
- 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 Gemcitabine
- 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 Leiomyosarcoma
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 TP53, RB1 and ATRX alterations, Gradeand size, Smooth muscle markers, Oestrogen and progesterone receptor in uterine tumours), 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 Uterine leiomyosarcoma, Retroperitoneal and intra-abdominal leiomyosarcoma, Vascular leiomyosarcoma.
- 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 surgical resection; radiotherapy for limb and trunk tumours; no morcellation of uterine masses that could be sarcoma; adjuvant chemotherapy not standard.
Metastatic, first line
- For my situation (metastatic, first line), which of the standard options do you recommend and why?Why: Guideline options include: Doxorubicin plus trabectedin (LMS-04) or doxorubicin alone; gemcitabine-docetaxel as an alternative, especially in uterine disease.
- Am I a candidate for Doxorubicin, Trabectedin, Gemcitabine or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Later lines
- For my situation (later lines), which of the standard options do you recommend and why?Why: Guideline options include: Trabectedin, pazopanib, dacarbazine-based regimens, aromatase inhibitors in hormone receptor-positive low-grade uterine disease; metastasectomy for limited lung spread.
- Am I a candidate for Trabectedin, Pazopanib, Letrozole (and other aromatase inhibitors), 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 “No targeted or immune therapy works in most patients”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Uterine tumours are still discovered by accident after fibroid surgery”. 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
7targets
3drugs
7companies
4Latest papers
topQuery for this cancer: (TITLE:"Leiomyosarcoma" OR ABSTRACT:"Leiomyosarcoma" OR TITLE:"LMS" OR ABSTRACT:"LMS" OR TITLE:"Uterine leiomyosarcoma" OR ABSTRACT:"Uterine leiomyosarcoma" OR TITLE:"Smooth muscle sarcoma" OR ABSTRACT:"Smooth muscle sarcoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Leiomyosarcoma, not a curated reading list.
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