ORPHA:2126
Solitary fibrous tumor
Also known as: SFT
Publications
8,882
Trials
5
Interventional, condition-specific
Researchers
1,131
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Clinical definition (Orphanet)
A rare soft tissue tumor characterized by a well-circumscribed mass potentially occurring at any anatomical site, histopathologically showing spindled to ovoid cells arranged around a branching and hyalinized vasculature and variable stromal collagen deposition. Immunohistochemistry reveals CD34 and/or STAT6 expression. NAB2-STAT6 gene fusions are pathognomonic for this tumor, which may be malignant or benign. Clinically, most patients present with a slow-growing, painless mass. Large tumors may cause paraneoplastic syndromes such as Doege-Potter syndrome, with the induction of severe or acromegaloid features.
How rare: How common this is has not been clearly measured.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0016238
- MeSH:D054364
- UMLS:C1266119
- NCIT:C7634
Additional Mondo synonyms (6)
localised fibrous mesothelioma · localised fibrous tumour · localized fibrous mesothelioma · localized fibrous tumor · solitary fibrous tumor · submesothelial fibroma
Research stages
Trial readiness signals
Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”
2/6 stages with a signal
An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
8,882 matched papers (5,188 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- Interventional trialPresent
5 matched on ClinicalTrials.gov
Biology
Genes and phenotypes
Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.
Do we know what causes it?
Not yet — the cause hasn't been pinned down in GenCC.
No strong gene–disease assertion joined for this Orphanet entity.
Phenotypes (Monarch / HPO)
Not enriched in this build — Monarch phenotype joins were not run for this record.
Animal models (Monarch / Alliance)
Not enriched in this build.
Literature
Is anyone studying this?
8,882
8,882 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.
8,882 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).
5,188 in the last 10 years · low confidence
Phrase hits: 8,882 · MeSH hits: 0
Who's working on it?
1,131
Distinct author names in 200 sampled papers — named people below.
Who's working on it?
People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.
- 01Chen X6 papers · 2026
Department of Neurosurgery, Chinese PLA General Hospital, Beijing, China.
Papers in Europe PMC - 02Wang Y6 papers · 2026
Department of Ophthalmology, Peking University Third Hospital, Beijing 100191, China.
Papers in Europe PMC - 03Li Y4 papers · 2026
Department of Bioengineering, University of Texas at Dallas, Richardson, TX, 75080, USA; Center for Systems Biology, University of Texas at Dallas, Richardson, TX, 75080, USA. Electronic address: yxl121030@utdallas.edu.
Papers in Europe PMC - 04Li Z4 papers · 2026
Department of Neurosurgery, The First Affiliated Hospital of Wannan Medical College, Yijishan Hospital of Wannan Medical College, Wuhu, China.
Papers in Europe PMC - 05Mondaza-Hernandez JL4 papers · 2026
Instituto de Investigacion Sanitaria Fundacion Jimenez Diaz (IIS/FJD; UAM), Madrid, Spain.
Papers in Europe PMC - 06Moura DS4 papers · 2026
Instituto de Investigacion Sanitaria Fundacion Jimenez Diaz (IIS/FJD; UAM), Madrid, Spain.
Papers in Europe PMC - 07Zhang Y4 papers · 2026
Department of Urology & Andrology The First Affiliated Hospital of Guizhou University of Traditional Chinese Medicine Guiyang China.
Papers in Europe PMC - 08Bayrak BY3 papers · 2026
Department of Pathology, Kocaeli University School of Medicine, Kocaeli, Türkiye.
Papers in Europe PMC - 09Bleris L3 papers · 2026
Department of Bioengineering, University of Texas at Dallas, Richardson, TX, 75080, USA; Center for Systems Biology, University of Texas at Dallas, Richardson, TX, 75080, USA; Department of Biological Sciences, University of Texas at Dallas, Richardson, TX, 75080, USA.
Papers in Europe PMC - 10Cui Y3 papers · 2025
Oncology Department, Hebei General Hospital, Shijiazhuang, Hebei, China.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
5
interventional trials for this specific condition
5 interventional trials matched this specific condition name; none in our sample are currently recruiting.
Data as of 27 July 2026
5 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 87.9th percentile).
low confidence · 87.9th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
5 interventional trials matched after quoted-phrase search and title/condition post-filter.
No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.
Observational and natural-history studies
3 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.
None of the matched observational studies is currently listed as recruiting.
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.
"Solitary fibrous tumor" OR "localised fibrous mesothelioma" OR "localised fibrous tumour" OR "localized fibrous mesothelioma" OR "localized fibrous tumor" OR "submesothelial fibroma"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Solitary fibrous tumor" OR "localised fibrous mesothelioma" OR "localised fibrous tumour" OR "localized fibrous mesothelioma" OR "localized fibrous tumor" OR "submesothelial fibroma"
Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 5 interventional · 3 observational · 0 expanded access. Only interventional studies enter the trial headline.
Query health: ok — strategies attempted: phrase; with hits: phrase
Run this search on ClinicalTrials.gov
Synonyms dropped by stoplist: SFT
Confidence reasoning
- Preferred label is multi-word and distinctive
- 1 synonym(s) dropped by stoplist (may under-count)
- No label/synonym collisions with other diseases in this corpus
- Publication count (8882) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity
Ingested 2026-07-26T19:12:00.578Z