ORPHA:79105
Myxofibrosarcoma
Also known as: Fibromyxosarcoma · Myxoid malignant fibrous histiocytoma
Publications
4,254
Trials
20
Interventional, condition-specific
Researchers
1,299
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Clinical definition (Orphanet)
A rare soft tissue sarcoma characterized by a malignant, fibroblastic lesion with variably myxoid stroma, pleomorphism, and a distinctively curvilinear vascular pattern. The majority of tumors arise in the limbs including the limb girdles, more often in dermal/subcutaneous tissues than in the underlying fascia and skeletal muscle, and usually present as a slowly growing, painless mass. Depth of the lesion and tumor grade do not influence the high rate of local recurrence, while the percentage of metastasis and tumor-associated mortality are much higher in deep-seated and high-grade neoplasms.
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:0019202
- UMLS:C3714524
- NCIT:C6496
Additional Mondo synonyms (5)
fibromyxosarcoma · myxofibrosarcoma · myxoid MFH · myxoid fibrous histiocytoma · myxoid malignant fibrous histiocytoma
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
4,254 matched papers (2,988 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
20 matched on ClinicalTrials.gov (7 recruiting in sample)
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?
4,254
4,254 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.
4,254 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).
2,988 in the last 10 years · low confidence
Phrase hits: 4,254 · MeSH hits: 0
Who's working on it?
1,299
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.
- 01Iwata S5 papers · 2025
Department of Musculoskeletal Oncology and Rehabilitation, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-Ku, Tokyo, 104-0045, Japan.
Papers in Europe PMC - 02Rutkowski P5 papers · 2026
Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 00-001 Warsaw, Poland.
Papers in Europe PMC - 03Czarnecka AM4 papers · 2026
Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 00-001 Warsaw, Poland.
Papers in Europe PMC - 04Kawai A4 papers · 2026
Department of Musculoskeletal Oncology, National Cancer Centre Hospital, Tokyo, Japan.
Papers in Europe PMC - 05Kondo T4 papers · 2026
Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-Ku, Tokyo, 104-0045, Japan. proteomebioinformatics@gmail.com.
Papers in Europe PMC - 06Spałek Mj4 papers · 2026
Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 00-001 Warsaw, Poland.
Papers in Europe PMC - 07Sun Y4 papers · 2026
Department of Pathology, The Second Xiangya Hospital, Central South University, Changsha, 410011, Hunan, China.
Papers in Europe PMC - 08Tysarowski A4 papers · 2026
Cancer Molecular and Genetic Diagnostics Laboratory, Maria Sklodowska-Curie National Research Institute of Oncology, 00-001 Warsaw, Poland.
Papers in Europe PMC - 09Adachi Y3 papers · 2025
Division of Rare Cancer Research, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-Ku, Tokyo, 104-0045, Japan.
Papers in Europe PMC - 10Bobak K3 papers · 2026
Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 00-001 Warsaw, Poland.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
20
interventional trials for this specific condition
20 interventional trials matched this specific condition name; 7 currently recruiting in our sample.
Data as of 27 July 2026
20 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 94.5th percentile).
low confidence · 94.5th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
20 interventional trials matched after quoted-phrase search and title/condition post-filter.
- NCT05711615·RECRUITING·Testing Low-Dose Common Chemotherapy (Liposomal Doxorubicin) in Combination With an Anti-Cancer Drug, Peposertib, in Advanced Sarcoma
Conditions: Metastatic Dedifferentiated Liposarcoma · Metastatic Leiomyosarcoma · Metastatic Myxofibrosarcoma · Metastatic Sarcoma·Matched via name phrase
- NCT07169344·RECRUITING·Hypofractionated, 3-week, Preoperative Proton or X-ray Radiotherapy for Patients With Localized Soft Tissue Sarcoma
Conditions: Soft Tissue Sarcoma (Excluding GIST) · Soft Tissue Sarcoma Adult · Soft Tissue Sarcoma of the Trunk and Extremities · Synovial Sarcomas·Matched via name phrase
- NCT04332874·RECRUITING·A Study of Pembrolizumab Plus Local Chemotherapy Using Isolated Limb Infusion (ILI) for Patients With Sarcoma in the Arm or Leg
Conditions: Sarcoma · Myxofibrosarcoma · Undifferentiated Pleomorphic Sarcoma · Alveolar Soft Part Sarcoma·Matched via name phrase
- NCT07173972·RECRUITING·Dose-escalated, Hypofractionated, Definitive Proton Radiotherapy for Patients With Inoperable Soft Tissue Sarcoma.
Conditions: Soft Tissue Sarcoma (STS) · Soft Tissue Sarcoma Adult · Soft Tissue Sarcoma of the Trunk and Extremities · Synovial Sarcomas·Matched via name phrase
- NCT06789172·RECRUITING·A Phase 1, First-in-human Study of OKN4395 and Pembrolizumab in Patients With Solid Tumors
Conditions: Solid Tumours · Sarcoma · HNSCC · Non Small Cell Lung Cancer·Matched via name phrase
- NCT06239272·RECRUITING·NRSTS2021, A Risk Adapted Study Evaluating Maintenance Pazopanib, Limited Margin, Dose-Escalated Radiation Therapy and Selinexor in Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS)
Conditions: Adipocytic Neoplasm · Liposarcoma · Atypical Fibroxanthoma · Angiomatoid Fibrous Histiocytoma·Matched via name phrase
- NCT06526897·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
Conditions: Adult Pleomorphic Rhabdomyosarcoma · AJCC Grade 2 Sarcoma · AJCC Grade 3 Sarcoma · Alveolar Soft Part Sarcoma·Matched via name phrase
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.
"Myxofibrosarcoma" OR "Fibromyxosarcoma" OR "Myxoid malignant fibrous histiocytoma" OR "myxoid MFH" OR "myxoid fibrous histiocytoma"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Myxofibrosarcoma" OR "Fibromyxosarcoma" OR "Myxoid malignant fibrous histiocytoma" OR "myxoid MFH" OR "myxoid fibrous histiocytoma"
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: 20 interventional · 0 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
Confidence reasoning
- Preferred label is short or not clearly distinctive
- No synonyms dropped by stoplist
- No label/synonym collisions with other diseases in this corpus
- Publication count (4254) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity
Ingested 2026-07-27T01:57:25.371Z
