Soft tissue sarcoma

Summary

Soft tissue sarcoma (MONDO:0018078) is a cancer (an umbrella term covering 46 Mondo subtypes) and 410 clinical trials. Top therapeutic interventions include docetaxel anhydrous, trabectedin, and pazopanib. A subtype of sarcoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Prevalence: 1-5 / 10 000 (Europe) [Orphanet-validated]
• Umbrella term: 46 Mondo subtypes
• Clinical trials: 410

Clinical features

Epidemiology

Prevalence records

3 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 100 000	4.74	Europe	Validated
Point prevalence	1-5 / 10 000	30	Europe	Validated
Annual incidence	1-9 / 100 000	3.6	France	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	soft tissue sarcoma
Mondo ID	MONDO:0018078
EFO	EFO:1001968
Orphanet	3394
DOID	DOID:0070663
NCIT	C9306
SNOMED CT	424952003
UMLS	C4551687
MedGen	1642116
GARD	0004898
NORD	1953
Is cancer (heuristic)	yes

Also known as: connective tissue sarcoma · malignant mesenchymal tumor · malignant mesenchymal tumour · malignant soft tissue tumor · malignant soft tissue tumour · non-Rhabdo. soft tissue sarcoma · sarcoma of soft tissue · sarcoma of the soft tissue · soft part sarcoma · soft tissue sarcoma

Data availability: 31 cell lines.

Disease family

This is a subtype of sarcoma. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › sarcoma › soft tissue sarcoma

Related subtypes (21): rectum sarcoma, ectomesenchymoma, spindle cell sarcoma, colon sarcoma, sarcomatosis, dendritic cell sarcoma, orbit sarcoma, sarcoma G1, uterine corpus sarcoma, giant cell tumor of soft tissue, lymphangiosarcoma, endometrioid stromal sarcoma, myeloid sarcoma, small cell sarcoma, chondrosarcoma, osteosarcoma, reticulum cell sarcoma, Ewing sarcoma, sarcoma of cervix uteri, mast cell sarcoma, bone sarcoma

Subtypes (46): esophagus sarcoma, bladder sarcoma, penile sarcoma, trachea sarcoma, retroperitoneal sarcoma, paranasal sinus sarcoma, pancreas sarcoma, vagina sarcoma, undifferentiated pleomorphic sarcoma, central nervous system sarcoma, ovarian sarcoma, liver sarcoma, lung sarcoma, laryngeal sarcoma, breast sarcoma, extraosseous osteosarcoma, rhabdoid tumor, mediastinum sarcoma, prostate sarcoma, gallbladder sarcoma, testis sarcoma, anus sarcoma, clear cell sarcoma, kidney sarcoma, thyroid sarcoma, heart sarcoma, small intestinal sarcoma, leiomyosarcoma, liposarcoma, fibrosarcoma, rhabdomyosarcoma, vulva sarcoma, intimal sarcoma, skin sarcoma, myxosarcoma, synovial sarcoma, alveolar soft part sarcoma, extraskeletal myxoid chondrosarcoma, angiosarcoma, epithelioid sarcoma, extraskeletal Ewing sarcoma, SMARCA4-deficient sarcoma of thorax, myxofibrosarcoma, desmoplastic small round cell tumor, undifferentiated round cell sarcoma, stromal sarcoma

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

Drugs indicated for this disease

2 approved, 35 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

Drug	Development status
Olaratumab	Approved (phase 4)
Trabectedin	Approved (phase 4)
Aldoxorubicin	Phase 3 (in late-stage trials)
Capmatinib	Phase 3 (in late-stage trials)
Carboplatin	Phase 3 (in late-stage trials)
Catequentinib	Phase 3 (in late-stage trials)
Ceritinib	Phase 3 (in late-stage trials)
Cisplatin	Phase 3 (in late-stage trials)
Dacarbazine	Phase 3 (in late-stage trials)
Dactinomycin	Phase 3 (in late-stage trials)
Dexamethasone	Phase 3 (in late-stage trials)
Doxorubicin	Phase 3 (in late-stage trials)
Epirubicin	Phase 3 (in late-stage trials)
Etoposide	Phase 3 (in late-stage trials)
Filgrastim	Phase 3 (in late-stage trials)
Futibatinib	Phase 3 (in late-stage trials)
Gemcitabine	Phase 3 (in late-stage trials)
Glasdegib	Phase 3 (in late-stage trials)
Idarubicin	Phase 3 (in late-stage trials)
Ifosfamide	Phase 3 (in late-stage trials)
Ipilimumab	Phase 3 (in late-stage trials)
Lapatinib	Phase 3 (in late-stage trials)
Melphalan	Phase 3 (in late-stage trials)
Nilotinib	Phase 3 (in late-stage trials)
Nivolumab	Phase 3 (in late-stage trials)
Onfekafusp Alfa	Phase 3 (in late-stage trials)
Paclitaxel	Phase 3 (in late-stage trials)
Palbociclib	Phase 3 (in late-stage trials)
Palifosfamide	Phase 3 (in late-stage trials)
Pazopanib	Phase 3 (in late-stage trials)
Pegargiminase	Phase 3 (in late-stage trials)
Prednisolone	Phase 3 (in late-stage trials)
Prednisone	Phase 3 (in late-stage trials)
Ridaforolimus	Phase 3 (in late-stage trials)
Sargramostim	Phase 3 (in late-stage trials)
Trametinib	Phase 3 (in late-stage trials)
Trofosfamide	Phase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): ANTINEOPLASTON A10, Amrubicin, Axitinib, Balstilimab, Bevacizumab, Bintrafusp Alfa, Bortezomib, Cabozantinib, Cadonilimab, Camrelizumab, Dexrazoxane, Durvalumab, Elesclomol, Endostatin, N-Terminal-Mggshhhhh, Envafolimab, Everolimus, Evofosfamide, Fluzoparib, Fruquintinib, Glufosfamide, Hafnium Oxide, Ibcasertib, Ixabepilone, Lurbinectedin, Olaparib, Pembrolizumab, Perifosine, Pirarubicin, Pomalidomide, Regorafenib, Relatlimab, Retifanlimab, Ribociclib, Rivoceranib, Selumetinib, Sintilimab, Sirolimus, Sorafenib, Sunitinib, Surufatinib, Talimogene Laherparepvec, Temozolomide, Temsirolimus, Thalidomide, Thiotepa, Tivozanib, Toripalimab, Trebananib, Tremelimumab, Vorinostat.

Clinical trials & evidence

Clinical trials

Clinical trials: 410.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	152
Not specified	100
PHASE1	75
PHASE1/PHASE2	49
PHASE3	17
EARLY_PHASE1	9
PHASE4	5
PHASE2/PHASE3	3

Top trials by phase / activity

NCT	Phase	Status	Title
NCT03244020	PHASE4	ENROLLING_BY_INVITATION	LMWH vs Aspirin for VTE Prophylaxis in Orthopaedic Oncology
NCT06506838	PHASE4	RECRUITING	Anlotinib Combined With Doxorubicin and Radiotherapy as Neoadjuvant Treatment for Soft Tissue Sarcoma
NCT03514381	PHASE4	COMPLETED	Pharmacologic Interaction Between Ifosfamide and Aprepitant in Treated Patients With Soft Tissue Sarcoma
NCT04223583	PHASE4	COMPLETED	Study on the Treatment of Soft Tissue Sarcoma With First-line Chemotherapy Failure by Anrotenil Hydrochloride Capsule
NCT04854018	PHASE4	COMPLETED	Indo-cyanine Green (ICG) in Paediatric Oncology MIS
NCT02180867	PHASE2/PHASE3	ACTIVE_NOT_RECRUITING	Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Before Surgery in Treating Patients With Newly Diagnosed Non-rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery
NCT03016819	PHASE3	RECRUITING	Phase III Trial of Anlotinib, Catequentinib in Advanced Alveolar Soft Part Sarcoma, Leiomyosarcoma, Synovial Sarcoma (APROMISS)
NCT04307277	PHASE3	RECRUITING	Interest of Peri Operative CHemotherapy In Patients With CINSARC High-risk Localized Soft Tissue Sarcoma
NCT05328258	PHASE3	RECRUITING	Use of GnRHa During Chemotherapy for Fertility Protection
NCT05597917	PHASE3	RECRUITING	tTF-NGR Randomized Study - STS
NCT05712694	PHASE3	RECRUITING	Study of ADI-PEG 20 or Placebo Plus Gem and Doc in Previously Treated Subjects With Leiomyosarcoma (ARGSARC)
NCT06332274	PHASE3	RECRUITING	tislelizUMaB in canceR Patients With molEcuLar residuaL Disease
NCT06988475	PHASE2/PHASE3	RECRUITING	DETERMINE Trial Treatment Arm 06: Capmatinib in Adult Patients With Cancers Harbouring MET Dysregulations
NCT00876031	PHASE3	COMPLETED	Trial for Localised High-risk Rhabdomyosarcoma and Rhabdomyosarcoma-like Soft Tissue Sarcoma
NCT01168791	PHASE3	COMPLETED	Study of Palifosfamide-tris in Combination With Doxorubicin in Patients With Front-line Metastatic Soft Tissue Sarcoma
NCT01327885	PHASE3	COMPLETED	Phase 3 Study to Compare the Efficacy and Safety of Eribulin With Dacarbazine in Subjects With Soft Tissue Sarcoma
NCT01440088	PHASE3	COMPLETED	A Trial of TH-302 in Combination With Doxorubicin Versus Doxorubicin Alone to Treat Patients With Locally Advanced Unresectable or Metastatic Soft Tissue Sarcoma
NCT02449343	PHASE2/PHASE3	UNKNOWN	Study of Anlotinib in Patients With Soft Tissue Sarcoma（STS）(ALTER0203)
NCT02451943	PHASE3	COMPLETED	A Study of Doxorubicin Plus Olaratumab (LY3012207) in Participants With Advanced or Metastatic Soft Tissue Sarcoma
NCT02672527	PHASE3	COMPLETED	Trial Comparing Trabectedin to the Best Supportive Care in Patients With Sarcoma
NCT03520959	PHASE3	TERMINATED	A Phase 3, Randomized, Double-blind, Placebo-controlled Study For Subjects With Locally-advanced Unresectable or Metastatic Synovial Sarcoma (V943-003, IMDZ-04-1702)
NCT03784014	PHASE3	COMPLETED	Molecular Profiling of Advanced Soft-tissue Sarcomas
NCT04650984	PHASE3	UNKNOWN	A Study Comparing the Efficacy of L19TNF+Doxorubicin vs Doxorubicin Alone as First-line Therapy in Patients With Advanced or Metastatic Soft Tissue Sarcoma
NCT05121350	PHASE3	UNKNOWN	A Clinical Trial to Evaluate the Efficacy and Safety of Anotinib Hydrochloride Capsule Combined With Epirubicin Hydrochloride Versus Placebo Combined With Epirubicin Hydrochloride in First-line Treatment of Advanced Soft Tissue Sarcoma
NCT06370871	PHASE3	WITHDRAWN	Brightline-3: A Study to Find Out Whether Brigimadlin in Combination With Ezabenlimab Helps People With Advanced Soft Tissue Sarcoma
NCT00186992	PHASE2	ACTIVE_NOT_RECRUITING	Radiation Therapy to Treat Musculoskeletal Tumors
NCT02867592	PHASE2	ACTIVE_NOT_RECRUITING	Cabozantinib-S-Malate in Treating Younger Patients With Recurrent, Refractory, or Newly Diagnosed Sarcomas, Wilms Tumor, or Other Rare Tumors
NCT02923778	PHASE2	ACTIVE_NOT_RECRUITING	Talimogene Laherparepvec and Radiation Therapy in Treating Patients With Newly Diagnosed Soft Tissue Sarcoma That Can Be Removed by Surgery
NCT03114527	PHASE2	ACTIVE_NOT_RECRUITING	Phase II Trial of Ribociclib and Everolimus in Advanced Dedifferentiated Liposarcoma (DDL) and Leiomyosarcoma (LMS)
NCT03138161	PHASE1/PHASE2	RECRUITING	SAINT:Trabectedin, Ipilimumab and Nivolumab for Previously Treated Advanced Soft Tissue Sarcoma
NCT03155620	PHASE2	ACTIVE_NOT_RECRUITING	Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders (The Pediatric MATCH Screening Trial)
NCT03213652	PHASE2	ACTIVE_NOT_RECRUITING	Ensartinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With ALK or ROS1 Genomic Alterations (A Pediatric MATCH Treatment Trial)
NCT03213704	PHASE2	ACTIVE_NOT_RECRUITING	Larotrectinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With NTRK Fusions (A Pediatric MATCH Treatment Trial)
NCT03449108	PHASE2	ACTIVE_NOT_RECRUITING	LN-145 or LN-145-S1 in Treating Patients With Relapsed or Refractory Ovarian Cancer, Triple Negative Breast Cancer (TNBC), Anaplastic Thyroid Cancer, Osteosarcoma, or Other Bone and Soft Tissue Sarcomas
NCT03698994	PHASE2	ACTIVE_NOT_RECRUITING	Ulixertinib in Treating Patients With Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With MAPK Pathway Mutations (A Pediatric MATCH Treatment Trial)
NCT03719430	PHASE2	ACTIVE_NOT_RECRUITING	APX005M and Doxorubicin in Advanced Sarcoma
NCT03899792	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	A Study of Oral LOXO-292 (Selpercatinib) in Pediatric Participants With Advanced Solid or Primary Central Nervous System (CNS) Tumors
NCT03965234	PHASE1/PHASE2	RECRUITING	Pulmonary Suffusion in Controlling Minimal Residual Disease in Patients With Sarcoma or Colorectal Metastases
NCT03972930	PHASE2	ACTIVE_NOT_RECRUITING	Hypofractionated Radiotherapy for Soft Tissue Sarcomas
NCT04028063	PHASE2	ACTIVE_NOT_RECRUITING	Doxorubicin Plus Dual Checkpoint Blockade for Soft Tissue Sarcomas

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
DOCETAXEL ANHYDROUS	4	22
TRABECTEDIN	4	12
PAZOPANIB	4	10
ERIBULIN	4	7
OLARATUMAB	4	7
DEXRAZOXANE	4	6
LAROTRECTINIB	4	4
TALIMOGENE LAHERPAREPVEC	4	4
ABEMACICLIB	4	3
RIBOCICLIB	4	3
SELPERCATINIB	4	3
AXITINIB	4	2
CAPMATINIB	4	2
ENOXAPARIN SODIUM	4	2
ENSARTINIB	4	2
IFOSFAMIDE	4	2
INDOCYANINE GREEN ACID FORM	4	2
IVOSIDENIB	4	2
LIFILEUCEL	4	2
LURBINECTEDIN	4	2
TAZEMETOSTAT	4	2
VEMURAFENIB	4	2
AVELUMAB	4	1
CABOZANTINIB	4	1
CABOZANTINIB S-MALATE	4	1
CERITINIB	4	1
DABRAFENIB	4	1
DACARBAZINE	4	1
DIGOXIN	4	1
DOSTARLIMAB	4	1