Myxofibrosarcoma

disease
On this page

Also known as fibromyxosarcomaMFSmyxoid fibrous histiocytomamyxoid malignant fibrous histiocytomamyxoid MFH

Summary

Myxofibrosarcoma (MONDO:0019202) is a disease and 20 clinical trials. Top therapeutic interventions include pazopanib, dexrazoxane, and ifosfamide. A subtype of soft tissue sarcoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

  • Prevalence: Unknown (Worldwide)
  • Clinical trials: 20

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

FieldValue
Canonical namemyxofibrosarcoma
Mondo IDMONDO:0019202
Orphanet79105
DOIDDOID:0080534
ICD-11405689402
NCITC6496
SNOMED CT253042009
UMLSC3714524
MedGen811359
GARD0018941
MedDRA10066948
Is cancer (heuristic)no

Also known as: fibromyxosarcoma · MFS · myxofibrosarcoma · myxoid fibrous histiocytoma · myxoid malignant fibrous histiocytoma · myxoid MFH

Data availability: 22 cell lines.

Disease family

This is a subtype of soft tissue 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 tumorneoplastic disease or syndromeneoplasmcancersarcomasoft tissue sarcomamyxofibrosarcoma

Related subtypes (45): 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, 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

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

Clinical trials & evidence

Clinical trials

Clinical trials: 20.

Phase distribution (across all retrieved trials)

PhaseTrials
PHASE29
PHASE15
PHASE1/PHASE23
PHASE2/PHASE31
PHASE31
Not specified1

Top trials by phase / activity

NCTPhaseStatusTitle
NCT02180867PHASE2/PHASE3ACTIVE_NOT_RECRUITINGRadiation 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
NCT06370871PHASE3WITHDRAWNBrightline-3: A Study to Find Out Whether Brigimadlin in Combination With Ezabenlimab Helps People With Advanced Soft Tissue Sarcoma
NCT04332874PHASE2RECRUITINGA Study of Pembrolizumab Plus Local Chemotherapy Using Isolated Limb Infusion (ILI) for Patients With Sarcoma in the Arm or Leg
NCT04480502PHASE2ACTIVE_NOT_RECRUITINGENVASARC: Envafolimab And Envafolimab With Ipilimumab In Patients With Undifferentiated Pleomorphic Sarcoma Or Myxofibrosarcoma
NCT06239272PHASE1/PHASE2RECRUITINGNRSTS2021, A Risk Adapted Study Evaluating Maintenance Pazopanib, Limited Margin, Dose-Escalated Radiation Therapy and Selinexor in Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS)
NCT07169344PHASE2RECRUITINGHypofractionated, 3-week, Preoperative Proton or X-ray Radiotherapy for Patients With Localized Soft Tissue Sarcoma
NCT07173972PHASE2RECRUITINGDose-escalated, Hypofractionated, Definitive Proton Radiotherapy for Patients With Inoperable Soft Tissue Sarcoma.
NCT01653028PHASE2COMPLETEDAlisertib in Treating Patients With Advanced or Metastatic Sarcoma
NCT02584309PHASE2COMPLETEDDoxorubicin With Upfront Dexrazoxane for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma
NCT02601209PHASE1/PHASE2TERMINATEDSapanisertib or Pazopanib Hydrochloride in Treating Patients With Locally Advanced or Metastatic Sarcoma
NCT03425279PHASE1/PHASE2COMPLETEDCAB-AXL-ADC Safety and Efficacy Study in Adult and Adolescent Patients With Sarcoma
NCT03651375PHASE2UNKNOWNHypofractionated Radiotherapy With Sequential Chemotherapy in Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall
NCT04906876PHASE2WITHDRAWNA Phase 2 Study of 9-ING-41Combined With Chemotherapy in Adolescents and Adults With Advanced Sarcomas
NCT05116800PHASE2WITHDRAWNPhase 2 Study of 9-ING-41 With Chemotherapy in Sarcoma
NCT04420975PHASE1ACTIVE_NOT_RECRUITINGNivolumab and BO-112 Before Surgery for the Treatment of Resectable Soft Tissue Sarcoma
NCT05711615PHASE1RECRUITINGTesting Low-Dose Common Chemotherapy (Liposomal Doxorubicin) in Combination With an Anti-Cancer Drug, Peposertib, in Advanced Sarcoma
NCT06789172PHASE1RECRUITINGA Phase 1, First-in-human Study of OKN4395 and Pembrolizumab in Patients With Solid Tumors
NCT03009201PHASE1COMPLETEDRibociclib and Doxorubicin in Treating Patients With Metastatic or Advanced Soft Tissue Sarcomas That Cannot Be Removed by Surgery
NCT04242238PHASE1COMPLETEDStudy of DCC-3014 in Combination With Avelumab in Patients With Advanced or Metastatic Sarcomas
NCT06526897Not specifiedNOT_YET_RECRUITINGEvaluation of Chest CT Versus Chest X-Ray for Lung Surveillance After Curative-Intent Resection of High-Risk Truncal-Extremity Soft Tissue Sarcoma

Drugs tested across these trials (top 30)

MoleculeMax phaseTrials referencing
PAZOPANIB44
DEXRAZOXANE43
IFOSFAMIDE42
DACTINOMYCIN41
SELINEXOR41
ALISERTIB31
BRIGIMADLIN31
ENVAFOLIMAB31
EZABENLIMAB31
VIMSELTINIB31
ELRAGLUSIB22
SAPANISERTIB21
EFROFILCON A11
NEDISERTIB11
CHEMBL406876801
CHEMBL417127701
CHEMBL396263201
CHEMBL399193301
CHEMBL474839101