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ORPHA:178342

Inflammatory myofibroblastic tumor

low confidenceDisorder

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.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

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.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    8,317 matched papers (4,398 in last 10 years) Source

  3. Phenotype characterisedNot found

    No HPO disease–phenotype associations via Monarch for these Mondo IDs

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. 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)

None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.

Open Targets candidates

3

Drugs / clinical candidates · MONDO_0015798

CTD chemicals (MyDisease.info)

1 associated chemical · 74 pathways. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Crizotinib · therapeutic

Pathways: RNA transport; Non-small cell lung cancer; Nuclear pore complex; ISG15 antiviral mechanism; Antiviral mechanism by IFN-stimulated genes; Cytokine Signaling in Immune system; Metabolism; Transport of the SLBP independent Mature mRNA

MyDisease.info · MONDO:0015798

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=3967) is 59.

8,317 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

4,398 in the last 10 years · low confidence

Phrase hits: 8,312 · MeSH hits: 9

Open Europe PMC search

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.

  1. 01
    Wang Y11 papers · 2026

    Department of Cardiovascular Surgery, Qinghai University Affiliated Hospital, Xining, China.

    Papers in Europe PMC
  2. 02
    Wang X4 papers · 2026

    Department of Thoracic Surgery, Huashan Hospital & Cancer Metastasis Institute, Fudan University, Shanghai, 200040, China.

    Papers in Europe PMC
  3. 03
    Chibwae A3 papers · 2026

    Department of Surgery, School of Medicine Muhimbili University of Health and Allied Sciences Dar es Salaam Tanzania.

    Papers in Europe PMC
  4. 04
    Li H3 papers · 2026

    Department of Pathology, Johns Hopkins Hospital, Baltimore, Maryland.

    Papers in Europe PMC
  5. 05
    Li 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
  6. 06
    Li W3 papers · 2025

    Department of Cardiovascular Medicine, The Affiliated Hospital of Guizhou Medical University, Guiyang, China.

    Papers in Europe PMC
  7. 07
    Li 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
  8. 08
    Li Y3 papers · 2026

    Department of Urology, The Affiliated Hospital of Guizhou Medical University, Guiyang, China.

    Papers in Europe PMC
  9. 09
    Liu H3 papers · 2026

    Department of Thoracic Surgery, Huashan Hospital & Cancer Metastasis Institute, Fudan University, Shanghai, 200040, China.

    Papers in Europe PMC
  10. 10
    Liu 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 11 September 2026

6 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 90.1th percentile).

low confidence · 90.1th 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.

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.

Open the complete matched search on ClinicalTrials.gov

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 0 · after dedupe 0 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 0 · dropped 0 · fetched 2026-07-30

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Inflammatory myofibroblastic tumor — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This 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"

Run this search on Europe PMC

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