ORPHA:178342
Inflammatory myofibroblastic tumor
Publications
8,317
Trials
6
Interventional, condition-specific
Researchers
1,072
Distinct authors in sample
Gene link
—
Readiness
2/6
Stages with a signal
Clinical definition (Orphanet)
Inflammatory myofibroblastic tumor is a rare neoplastic lesion of the submucosal stroma, which can develop in any organ, often occurring in the lung, mesentery, omentum and the retroperitoneal region. It is histologically heterogenous, composed of spindle-shaped cells, myofibroblasts and inflammatory cells. It is usually benign, however local invasion, recurrence, malignant transformation with vascular invasion and metastases may occur. The presentation is nonspecific and depends on the organ involved. Some patients may present with paraneoplastic syndrome (fever, malaise, weight loss, anemia, thrombocytosis) or symptoms related to compression of adjacent organs, such as bowel obstruction.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0015798
- MeSH:D006104
- UMLS:C0334121
- NCIT:C6481
Additional Mondo synonyms (5)
IMT · inflammatory fibrosarcoma · inflammatory myofibroblastic neoplasm · inflammatory myofibroblastic tumor · inflammatory pseudotumor
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
8,317 matched papers (4,398 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
6 matched on ClinicalTrials.gov (1 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?
8,317
8,317 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.
8,317 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).
4,398 in the last 10 years · low confidence
Phrase hits: 8,312 · MeSH hits: 9
Who's working on it?
1,072
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.
- 01Wang Y11 papers · 2026
Department of Cardiovascular Surgery, Qinghai University Affiliated Hospital, Xining, China.
Papers in Europe PMC - 02Wang X4 papers · 2026
Department of Thoracic Surgery, Huashan Hospital & Cancer Metastasis Institute, Fudan University, Shanghai, 200040, China.
Papers in Europe PMC - 03Chibwae A3 papers · 2026
Department of Surgery, School of Medicine Muhimbili University of Health and Allied Sciences Dar es Salaam Tanzania.
Papers in Europe PMC - 04Li H3 papers · 2026
Department of Pathology, Johns Hopkins Hospital, Baltimore, Maryland.
Papers in Europe PMC - 05Li Q3 papers · 2026
Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College.
Papers in Europe PMC - 06Li W3 papers · 2025
Department of Cardiovascular Medicine, The Affiliated Hospital of Guizhou Medical University, Guiyang, China.
Papers in Europe PMC - 07Li X3 papers · 2026
Department of Pulmonary and Critical Care Medicine, Division of Pulmonary Diseases, State Key Laboratory of Biotherapy, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, Sichuan, China.
Papers in Europe PMC - 08Li Y3 papers · 2026
Department of Urology, The Affiliated Hospital of Guizhou Medical University, Guiyang, China.
Papers in Europe PMC - 09Liu H3 papers · 2026
Department of Thoracic Surgery, Huashan Hospital & Cancer Metastasis Institute, Fudan University, Shanghai, 200040, China.
Papers in Europe PMC - 10Liu Y3 papers · 2026
Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
6
interventional trials for this specific condition
6 interventional trials matched this specific condition name; 1 currently recruiting in our sample.
Data as of 27 July 2026
6 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 89th percentile).
low confidence · 89th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
6 interventional trials matched after quoted-phrase search and title/condition post-filter.
- NCT04925609·RECRUITING·Brigatinib in Pediatric and Young Adult Patients With ALK+ ALCL, IMT or Other Solid Tumors
Conditions: Anaplastic Large Cell Lymphoma, ALK-Positive · Inflammatory Myofibroblastic Tumor · Other Solid Tumor·Matched via name phrase
Observational and natural-history studies
1 observational study matches 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.
"Inflammatory myofibroblastic tumor" OR "inflammatory fibrosarcoma" OR "inflammatory myofibroblastic neoplasm" OR "inflammatory pseudotumor"
MeSH descriptor terms unioned into the query: Granuloma, Plasma Cell
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Inflammatory myofibroblastic tumor" OR "inflammatory fibrosarcoma" OR "inflammatory myofibroblastic neoplasm" OR "inflammatory pseudotumor" OR "Granuloma, Plasma Cell"
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: 6 interventional · 1 observational · 0 expanded access. Only interventional studies enter the trial headline.
Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh
Run this search on ClinicalTrials.gov
Synonyms dropped by stoplist: IMT
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 (8317) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity
Ingested 2026-07-27T08:49:00.977Z