Fibrosarcoma

Summary

Fibrosarcoma (MONDO:0005164) is a disease (an umbrella term covering 11 Mondo subtypes) and 16 clinical trials. Top therapeutic interventions include dexrazoxane, ifosfamide, and pazopanib. A subtype of fibroblastic neoplasm — 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]
• Umbrella term: 11 Mondo subtypes
• Clinical trials: 16

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000	0.01	Europe	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	fibrosarcoma
Mondo ID	MONDO:0005164
EFO	EFO:0002087
MeSH	D005354
Orphanet	2030
DOID	DOID:3355
NCIT	C3043
SNOMED CT	443250000
UMLS	C0016057
MedGen	5178
GARD	0002327
MedDRA	10016632
Is cancer (heuristic)	no

Also known as: fibrocytic tumor · fibrocytic tumour · fibrosarcoma · fibrosarcoma (disease) · fibrosarcoma (excluding infantile fibrosarcoma) · fibrosarcoma - not infantile · fibrosarcoma, malignant · malignant fibromatous neoplasm

Data availability: 1 HPO phenotype · 109 cell lines.

Disease family

An umbrella term covering 11 Mondo subtypes.

Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › mesenchymal cell neoplasm › fibroblastic neoplasm › fibrosarcoma

Related subtypes (6): nodular fasciitis, fibromatosis, fibroma, solitary fibrous tumor, elastofibroma dorsi, myofibroblastoma

Subtypes (11): bone fibrosarcoma, conventional fibrosarcoma, pediatric fibrosarcoma, kidney fibrosarcoma, breast fibrosarcoma, heart fibrosarcoma, central nervous system fibrosarcoma, small intestinal fibrosarcoma, liver fibrosarcoma, low grade fibromyxoid sarcoma, dermatofibrosarcoma protuberans

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

0 approved, 1 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

Drug	Development status
Ifosfamide	Phase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Bevacizumab.

Clinical trials & evidence

Clinical trials

Clinical trials: 16.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	10
Not specified	3
PHASE2/PHASE3	1
PHASE1/PHASE2	1
PHASE1	1

Top trials by phase / activity

NCT	Phase	Status	Title
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
NCT06277154	PHASE2	RECRUITING	MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment of Advanced Soft Tissue Sarcoma
NCT06638931	PHASE2	RECRUITING	Agnostic Therapy in Rare Solid Tumors
NCT06849986	PHASE2	RECRUITING	IO Combined With AI as First-line Treatment for Patients With Soft Tissue Sarcoma(TAIS)
NCT00141765	PHASE2	COMPLETED	Study of High-Dose Chemotherapy With Bone Marrow or Stem Cell Transplant for Rare Poor-Prognosis Cancers
NCT00356031	PHASE2	COMPLETED	Bevacizumab and Radiation Therapy for Sarcomas
NCT00400569	PHASE2	COMPLETED	Phase II Study of Sunitinib Malate for Metastatic and/or Surgically Unresectable Soft Tissue Sarcoma
NCT00503295	PHASE2	COMPLETED	Safety and Efficacy Study of REOLYSIN® in the Treatment of Bone and Soft Tissue Sarcomas Metastatic to the Lung
NCT01962103	PHASE1/PHASE2	COMPLETED	Study to Find a Safe Dose and Show Early Clinical Activity of Weekly Nab-paclitaxel in Pediatric Patients With Recurrent/ Refractory Solid Tumors
NCT02584309	PHASE2	COMPLETED	Doxorubicin With Upfront Dexrazoxane for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma
NCT03651375	PHASE2	UNKNOWN	Hypofractionated Radiotherapy With Sequential Chemotherapy in Marginally Resectable Soft Tissue Sarcomas of Extremities or Trunk Wall
NCT03989596	PHASE2	UNKNOWN	Hypofractionated Radiotherapy With Hyperthermia in Unresectable or Marginally Resectable Soft Tissue Sarcomas
NCT03009201	PHASE1	COMPLETED	Ribociclib and Doxorubicin in Treating Patients With Metastatic or Advanced Soft Tissue Sarcomas That Cannot Be Removed by Surgery
NCT06387485	Not specified	RECRUITING	A Study to Evaluate the Utilization of 3D Printed Models in Pre-Operative Planning
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
NCT06662734	Not specified	COMPLETED	Infantile Versus Adult-type Fibrosarcoma and the Risk of Multiple Primary Malignancies

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
DEXRAZOXANE	4	3
IFOSFAMIDE	4	3
PAZOPANIB	4	3
SUNITINIB MALATE	4	1
CHEMBL4068768	0	1
CHEMBL4171277	0	1
CHEMBL3109278	0	1