Solitary fibrous tumor

Summary

Solitary fibrous tumor (MONDO:0016238) is a cancer (an umbrella term covering 5 Mondo subtypes) and 7 clinical trials. Top therapeutic interventions include trabectedin, axitinib, and eribulin. A subtype of fibroblastic neoplasm — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Prevalence: Unknown (Worldwide)
• Umbrella term: 5 Mondo subtypes
• Phenotypes (HPO): 29
• Clinical trials: 7

Clinical features

Signs & symptoms

Clinical features (HPO)

29 HPO clinical features (Orphanet curated; top 29 by frequency):

HPO ID	Term	Frequency
HP:0001824	Weight loss	Occasional (5-29%)
HP:0002664	Neoplasm	Occasional (5-29%)
HP:0010787	Genital neoplasm	Occasional (5-29%)
HP:0012378	Fatigue	Occasional (5-29%)
HP:0031459	Soft tissue neoplasm	Occasional (5-29%)
HP:0100527	Neoplasia of the pleura	Occasional (5-29%)
HP:0000016	Urinary retention	Very rare (<1-4%)
HP:0000290	Abnormality of the forehead	Very rare (<1-4%)
HP:0000651	Diplopia	Very rare (<1-4%)
HP:0001943	Hypoglycemia	Very rare (<1-4%)
HP:0001945	Fever	Very rare (<1-4%)
HP:0001988	Recurrent hypoglycemia	Very rare (<1-4%)
HP:0002019	Constipation	Very rare (<1-4%)
HP:0002585	Abnormality of the peritoneum	Very rare (<1-4%)
HP:0002896	Neoplasm of the liver	Very rare (<1-4%)
HP:0003419	Low back pain	Very rare (<1-4%)
HP:0004375	Neoplasm of the nervous system	Very rare (<1-4%)
HP:0004912	Hypophosphatemic rickets	Very rare (<1-4%)
HP:0007185	Loss of consciousness	Very rare (<1-4%)
HP:0008775	Abnormal prostate morphology	Very rare (<1-4%)
HP:0010784	Uterine neoplasm	Very rare (<1-4%)
HP:0012125	Prostate cancer	Very rare (<1-4%)
HP:0030166	Night sweats	Very rare (<1-4%)
HP:0030795	Reduced C-peptide level	Very rare (<1-4%)
HP:0031501	Pelvic mass	Very rare (<1-4%)
HP:0040216	Hypoinsulinemia	Very rare (<1-4%)
HP:0045026	Abnormality of the mediastinum	Very rare (<1-4%)
HP:0100526	Neoplasm of the lung	Very rare (<1-4%)
HP:0100650	Vaginal neoplasm	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	solitary fibrous tumor
Mondo ID	MONDO:0016238
MeSH	D054364
Orphanet	2126
NCIT	C7634
UMLS	C1266119
MedGen	224764
GARD	0015014
MedDRA	10018825
Is cancer (heuristic)	yes

Also known as: localised fibrous mesothelioma · localised fibrous tumour · localized fibrous mesothelioma · localized fibrous tumor · SFT · solitary fibrous tumor · solitary fibrous tumor/hemangiopericytoma · submesothelial fibroma

Data availability: 6 cell lines.

Disease family

An umbrella term covering 5 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 › solitary fibrous tumor

Related subtypes (6): nodular fasciitis, fibromatosis, fibrosarcoma, fibroma, elastofibroma dorsi, myofibroblastoma

Subtypes (5): liver solitary fibrous tumor, dedifferentiated solitary fibrous tumor, hemangiopericytoma, malignant, pleural solitary fibrous tumor, peritoneal solitary fibrous tumor

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

No approved or late-stage (phase ≥3) drug is indicated for this disease; the following are in earlier-phase trials only.

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

Clinical trials & evidence

Clinical trials

Clinical trials: 7.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	4
PHASE1	2
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT02066285	PHASE2	COMPLETED	Trial of Pazopanib in Patients With Solitary Fibrous Tumor and Extraskeletal Myxoid Chondrosarcoma
NCT02261207	PHASE2	COMPLETED	Phase II Study on Axitinib in Advanced Solitary Fibrous Tumor
NCT03023124	PHASE2	COMPLETED	Study With Trabectedin Versus Adriamycin Plus Dacarbazine, in Patients With Advanced Solitary Fibrous Tumor
NCT03840772	PHASE2	COMPLETED	Eribulin in Advanced Solitary Fibrous Tumor
NCT06789172	PHASE1	RECRUITING	A Phase 1, First-in-human Study of OKN4395 and Pembrolizumab in Patients With Solid Tumors
NCT07118176	PHASE1	RECRUITING	Determining the Biodistribution of an Imaging Tracer (68Ga-FAPi-46) in Patients With Solid Tumors or Hematologic Cancers
NCT04008238	Not specified	COMPLETED	Prospective Identification of Predictive Biomarkers of Trabectedin Efficacy in Non-L Soft-tissue Sarcoma Patients

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
TRABECTEDIN	4	2
AXITINIB	4	1
ERIBULIN	4	1
PAZOPANIB	4	1
FAPI-46 GA-68	2	1
CHEMBL4068768	0	1
CHEMBL4171277	0	1
CHEMBL3939307	0	1