Dedifferentiated liposarcoma
diseaseOn this page
Also known as DDLPSDDLS
Summary
Dedifferentiated liposarcoma (MONDO:0020563) is a disease and 26 clinical trials. Top therapeutic interventions include abemaciclib, retifanlimab, and cabazitaxel. A subtype of liposarcoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: 1-9 / 1 000 000 (Europe) [Orphanet-validated]
- Clinical trials: 26
Clinical features
Epidemiology
Prevalence records
1 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.27 | Europe | Validated |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | dedifferentiated liposarcoma |
| Mondo ID | MONDO:0020563 |
| EFO | EFO:0003085 |
| Orphanet | 99970 |
| DOID | DOID:0080531 |
| NCIT | C3704 |
| SNOMED CT | 404072004 |
| UMLS | C0205824 |
| MedGen | 60028 |
| GARD | 0019720 |
| Is cancer (heuristic) | no |
Also known as: DDLPS · DDLS · dedifferentiated liposarcoma
Data availability: 33 cell lines.
Disease family
This is a subtype of liposarcoma. 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 › lipomatous cancer › liposarcoma › dedifferentiated liposarcoma
Related subtypes (18): liposarcoma of bone, adult liposarcoma, esophagus liposarcoma, pediatric liposarcoma, larynx liposarcoma, liposarcoma of the ovary, fibroblastic liposarcoma, kidney liposarcoma, gastric liposarcoma, breast liposarcoma, mixed liposarcoma, vulvar liposarcoma, cutaneous liposarcoma, mediastinum liposarcoma, intracranial liposarcoma, well-differentiated liposarcoma, myxoid/round cell liposarcoma, pleomorphic liposarcoma
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
1 approved, 3 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.
| Drug | Development status |
|---|---|
| Trabectedin | Approved (phase 4) |
| Abemaciclib | Phase 3 (in late-stage trials) |
| Brigimadlin | Phase 3 (in late-stage trials) |
| Milademetan | Phase 3 (in late-stage trials) |
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Cabazitaxel, Cemiplimab, Doxorubicin, Ipilimumab, Nivolumab, Palbociclib, Pemigatinib, Pioglitazone, Plitidepsin, Retifanlimab, Ribociclib, Selinexor.
Clinical trials & evidence
Clinical trials
Clinical trials: 26.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 13 |
| PHASE1 | 4 |
| PHASE3 | 3 |
| PHASE1/PHASE2 | 3 |
| Not specified | 2 |
| PHASE2/PHASE3 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT04967521 | PHASE3 | ACTIVE_NOT_RECRUITING | SARC041: Study of Abemaciclib Versus Placebo in Patients with Advanced Dedifferentiated Liposarcoma |
| NCT06422806 | PHASE3 | RECRUITING | Measuring if Immunotherapy Plus Chemotherapy is Better Than Chemotherapy Alone for Patients With Aggressive Poorly Differentiated Sarcomas |
| NCT02606461 | PHASE2/PHASE3 | COMPLETED | Selinexor in Advanced Liposarcoma |
| NCT04979442 | PHASE3 | TERMINATED | Treatment of Milademetan Versus Trabectedin in Patient With Dedifferentiated Liposarcoma |
| NCT02846987 | PHASE2 | ACTIVE_NOT_RECRUITING | Study of Abemaciclib in Dedifferentiated Liposarcoma |
| NCT03307616 | PHASE2 | ACTIVE_NOT_RECRUITING | Nivolumab With and Without Ipilimumab and Radiation Therapy in Treating Patients With Recurrent or Resectable Undifferentiated Pleomorphic Sarcoma or Dedifferentiated Liposarcoma Before Surgery |
| NCT04438824 | PHASE2 | ACTIVE_NOT_RECRUITING | Palbociclib and INCMGA00012 in People With Advanced Liposarcoma |
| NCT04668300 | PHASE2 | ACTIVE_NOT_RECRUITING | Oleclumab and Durvalumab for the Treatment of Recurrent, Refractory, or Metastatic Sarcoma |
| NCT05694871 | PHASE2 | ACTIVE_NOT_RECRUITING | Testing the Addition of Cemiplimab to Palbociclib for the Treatment of Advanced Dedifferentiated Liposarcoma |
| NCT05886634 | PHASE2 | ACTIVE_NOT_RECRUITING | A Study of Etrumadenant and Zimberelimab in People With Dedifferentiated Liposarcoma |
| NCT06389799 | PHASE2 | RECRUITING | A Phase 2, Open Label Study of PEmigatinib and REtifanlimab in Advanced Dedifferentiated LIposarcoma (PERELI) |
| NCT06498648 | PHASE1/PHASE2 | RECRUITING | Testing the Addition of an Anti-cancer Drug, Abemaciclib, to the Usual Chemotherapy Treatment (Gemcitabine) for Soft Tissue Sarcoma |
| NCT06694324 | PHASE2 | NOT_YET_RECRUITING | First-line Treatment of Advanced/unresectable DDLPS |
| NCT06843967 | PHASE1/PHASE2 | RECRUITING | A Study of Mirdametinib in Combination With Palbociclib in People With Liposarcoma |
| NCT06975293 | PHASE1/PHASE2 | RECRUITING | STC-15 as a Part of Combination Therapy With Toripalimab in Selected Advanced Cancers and as Monotherapy in Participants With Selected Sarcomas |
| NCT00969917 | PHASE2 | WITHDRAWN | Study Evaluating the Efficacy and Safety of IPI 504 in Patients With Advanced Dedifferentiated Liposarcoma |
| NCT01913652 | PHASE2 | COMPLETED | Ph II Cabazitaxel DD Liposarcoma |
| NCT02500797 | PHASE2 | COMPLETED | Nivolumab With or Without Ipilimumab in Treating Patients With Metastatic Sarcoma That Cannot Be Removed by Surgery |
| NCT03651375 | PHASE2 | UNKNOWN | Hypofractionated Radiotherapy With Sequential Chemotherapy in Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall |
| NCT05496569 | PHASE2 | TERMINATED | TQB3616 Capsules in the Treatment of Dedifferentiated Liposarcoma |
| NCT05711615 | PHASE1 | RECRUITING | Testing Low-Dose Common Chemotherapy (Liposomal Doxorubicin) in Combination With an Anti-Cancer Drug, Peposertib, in Advanced Sarcoma |
| NCT06789172 | PHASE1 | RECRUITING | A Phase 1, First-in-human Study of OKN4395 and Pembrolizumab in Patients With Solid Tumors |
| NCT03604783 | PHASE1 | TERMINATED | Phase 1, First-in-human Study of Oral TP-1287 in Patients with Advanced Solid Tumors |
| NCT04242238 | PHASE1 | COMPLETED | Study of DCC-3014 in Combination With Avelumab in Patients With Advanced or Metastatic Sarcomas |
| NCT06526897 | Not specified | NOT_YET_RECRUITING | Evaluation of Chest CT Versus Chest X-Ray for Lung Surveillance After Curative-Intent Resection of High-Risk Truncal-Extremity Soft Tissue Sarcoma |
| NCT06115681 | Not specified | COMPLETED | Real-world Study of Dedifferentiated Liposarcoma Patients in China |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| ABEMACICLIB | 4 | 2 |
| RETIFANLIMAB | 4 | 2 |
| CABAZITAXEL | 4 | 1 |
| CEMIPLIMAB | 4 | 1 |
| PEMIGATINIB | 4 | 1 |
| SELINEXOR | 4 | 1 |
| TRABECTEDIN | 4 | 1 |
| RETASPIMYCIN | 3 | 2 |
| MILADEMETAN | 3 | 1 |
| OLECLUMAB | 3 | 1 |
| VIMSELTINIB | 3 | 1 |
| ZIMBERELIMAB | 3 | 1 |
| ETRUMADENANT | 2 | 1 |
| MIRDAMETINIB | 2 | 1 |
| NEDISERTIB | 1 | 1 |
| TP-1287 | 1 | 1 |
| CHEMBL5190225 | 0 | 2 |