Pleomorphic liposarcoma

Summary

Pleomorphic liposarcoma (MONDO:0020562) is a disease and 11 clinical trials. Top therapeutic interventions include ipilimumab, trabectedin, and mirdametinib. A subtype of liposarcoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: <1 / 1 000 000 (Europe) [Orphanet-validated]
• Phenotypes (HPO): 2
• Clinical trials: 11

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000	0.05	Europe	Validated

Signs & symptoms

Clinical features (HPO)

2 HPO clinical features (Orphanet curated; top 2 by frequency):

HPO ID	Term	Frequency
HP:0012034	Liposarcoma	Obligate (100%)
HP:0001482	Subcutaneous nodule	Very frequent (80-99%)

Identifiers

Disease identifiers

Field	Value
Canonical name	pleomorphic liposarcoma
Mondo ID	MONDO:0020562
EFO	EFO:0003083
Orphanet	99969
DOID	DOID:5702
NCIT	C3705
SNOMED CT	404071006
UMLS	C0205825
MedGen	61457
GARD	0019719
Is cancer (heuristic)	no

Also known as: pleomorphic liposarcoma · pleomorphic liposarcoma (morphologic abnormality) · PLLS · PLS

Data availability: 4 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 › pleomorphic 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, dedifferentiated 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

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 11.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	5
PHASE2	4
PHASE1/PHASE2	2

Top trials by phase / activity

NCT	Phase	Status	Title
NCT02275286	PHASE1/PHASE2	RECRUITING	Trabectedin Plus Radiotherapy in Soft Tissue Sarcoma Patients
NCT06843967	PHASE1/PHASE2	RECRUITING	A Study of Mirdametinib in Combination With Palbociclib in People With Liposarcoma
NCT06849986	PHASE2	RECRUITING	IO Combined With AI as First-line Treatment for Patients With Soft Tissue Sarcoma(TAIS)
NCT07173972	PHASE2	RECRUITING	Dose-escalated, Hypofractionated, Definitive Proton Radiotherapy for Patients With Inoperable Soft Tissue Sarcoma.
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
NCT05271435	Not specified	ACTIVE_NOT_RECRUITING	Digital Tools for Assessment of Motor Functions and Falls in ALS
NCT05966038	Not specified	RECRUITING	ALS/MND Natural History Study Data Repository
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
NCT01459302	Not specified	WITHDRAWN	Genetic Study of Familial and Sporadic ALS/Motor Neuron Disease, Miyoshi Myopathy and Other Neuromuscular Disorders
NCT02983539	Not specified	UNKNOWN	Detection of Circulating Tumor Cells in Patients With Sarcomas

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
IPILIMUMAB	4	1
TRABECTEDIN	4	1
MIRDAMETINIB	2	1
EFROFILCON A	1	1