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ORPHA:50251

Pleural mesothelioma

high confidenceDisorder

Query health: suspect — Only one of 2 strategies returned hits (phrase).

Publications

41,831

98.8th percentile

Trials

310

Interventional, condition-specific

Researchers

1,443

Distinct authors in sample

Gene link

—

Readiness

5/6

Stages with a signal

Clinical definition (Orphanet)

A rare, aggressive respiratory tumor arising from mesothelial cells of the pleura characterized by chest pain, dyspnea, cough and fatigue. It is strongly associated with asbestos exposure and typically develops after a long latency period (20-40 years). It usually presents as a diffuse invasive tumor but may also occur as a localized mass or as pleural mesothelioma in situ, a preinvasive lesion confined to the pleural surface.

How rare: 1-9 / 100 000 — about one to nine people per hundred thousand.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (12)

malignant mesothelial tumor · malignant mesothelial tumour · malignant mesothelioma · malignant mesothelioma (disease) · malignant neoplasm of mesothelium · malignant neoplasm of the mesothelium · malignant tumor of mesothelium · malignant tumor of the mesothelium · malignant tumour of mesothelium · malignant tumour of the mesothelium · mesothelioma, malignant · mesothelioma, somatic

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.”

5/6 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

    41,831 matched papers (23,914 in last 10 years) Source

  3. Phenotype characterisedPresent

    29 HPO annotations (e.g. Cough; Constitutional symptom; Chest pain) Source

  4. Animal modelPresent

    5 genotype models (Mus musculus) Source

  5. Orphan designationPresent

    5 FDA · 15 EMA designations (5 FDA orphan-indication approvals) — e.g. Nadofaragene firadenovec Source

  6. Interventional trialPresent

    310 matched on ClinicalTrials.gov (46 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)

29

Associated phenotypes · MONDO:0006292

  • Cough
  • Constitutional symptom
  • Chest pain
  • Dysphagia
  • Abnormal lung morphology

Showing 5 of 29 — open Monarch for the full list.

Animal models (Monarch / Alliance)

5

Model associations linked to this Mondo ID

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)

20

Designations · 5 with FDA orphan-indication approval

  • FDA Nadofaragene firadenovecMalignant mesothelioma · 2020-07-07 · Not FDA Approved for Orphan Indication
  • FDA tremelimumabMalignant mesothelioma · 2015-03-18 · Not FDA Approved for Orphan Indication
  • FDA porfimer sodiumMalignant mesothelioma · 2011-12-02 · Not FDA Approved for Orphan Indication
  • FDA CT-2584 mesylateMalignant mesothelioma · 1999-04-16 · Not FDA Approved for Orphan Indication
  • FDA 5,6-dihydro-5-azacytidineMalignant mesothelioma · 1992-05-11 · Not FDA Approved for Orphan Indication
  • EMA cisplatinTreatment of malignant mesothelioma · 29/08/2016 · PositiveEMA designation
  • EMA Tyr-Met-Phe-Pro-Asn-Ala-Pro-Tyr-Leu, Ser-Gly-Gln-Ala-Tyr-Met-Phe-Pro-Asn-Ala-Pro-Tyr-Leu-Pro-Ser-Cys-Leu-Glu-Ser, Arg-Ser-Asp-Glu-Leu-Val-Arg-His-His-Asn-Met-His-Gln-Arg-Asn-Met-Thr-Lys-Leu and Pro-Gly-Cys-Asn-Lys-Arg-Tyr-Phe-Lys-Leu-Ser-His-Leu-Gln-Met-His-Ser-Arg-Lys-His-Thr-GlyTreatment of malignant mesothelioma · 28/04/2016 · PositiveEMA designation
  • EMA paclitaxel obaluronateTreatment of malignant mesothelioma · 25/07/2023 · PositiveEMA designation

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

37

Drugs / clinical candidates · MONDO_0006292

CTD chemicals (MyDisease.info)

12 associated chemicals · 852 pathways. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Arsenic Trioxide · therapeutic
  • Clofarabine · therapeutic
  • Interleukin 1 Receptor Antagonist Protein · therapeutic
  • Itraconazole · therapeutic
  • Resveratrol · therapeutic
  • 8-Hydroxy-2'-Deoxyguanosine · marker/mechanism
  • Asbestos · marker/mechanism
  • Asbestos, Amosite · marker/mechanism
  • Asbestos, Amphibole · marker/mechanism
  • Asbestos, Crocidolite · marker/mechanism
  • Asbestos, Serpentine · marker/mechanism
  • fluor-edenite · marker/mechanism

Pathways: Caffeine metabolism; Lysine degradation; Tyrosine metabolism; Phenylalanine metabolism; Glutathione metabolism; Inositol phosphate metabolism; Porphyrin and chlorophyll metabolism; Metabolism of xenobiotics by cytochrome P450

MyDisease.info · MONDO:0006292

Literature

Is anyone studying this?

41,831

41,831 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.

41,831 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).

23,914 in the last 10 years · high confidence · 98.8th percentile (publications denominator)

Phrase hits: 41,831 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,443

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
    Baas P4 papers · 2026

    Department of Thoracic Oncology, Netherlands Cancer Institute, Amsterdam, Netherlands.

    Papers in Europe PMC
  2. 02
    Husain AN4 papers · 2026

    Department of Pathology, University of Chicago Medicine, Chicago, Illinois.

    Papers in Europe PMC
  3. 03
    Liu X4 papers · 2026

    Department of Pulmonary and Critical Care Medicine, Second People's Hospital of Gansu Province, Lanzhou, People's Republic of China.

    Papers in Europe PMC
  4. 04
    Wang Y4 papers · 2026

    Department of General Surgery, Second Xiangya Hospital, Central South University, Changsha, Hunan Province, China. 228202102@csu.edu.cn.

    Papers in Europe PMC
  5. 05
    Zucali PA4 papers · 2026

    IRCCS Humanitas Research Hospital, Humanitas Cancer Center, Rozzano, Milan, Italy.

    Papers in Europe PMC
  6. 06
    Blanquart C3 papers · 2026

    Nantes Université, Inserm UMR 1307, CNRS UMR 6075, Université d'Angers, CRCI2NA, Nantes, France.

    Papers in Europe PMC
  7. 07
    Burgers JA3 papers · 2026

    Department of Thoracic Oncology, Netherlands Cancer Institute, Amsterdam, Netherlands.

    Papers in Europe PMC
  8. 08
    Calabrese F3 papers · 2026

    Department of Cardiac, Thoracic, Vascular Sciences, and Public Health, University of Padova, University Hospital of Padova, Padova, Italy.

    Papers in Europe PMC
  9. 09
    Fennell DA3 papers · 2026

    MRC Toxicology Unit, University of Leicester and Leicester University Hospitals, Leicester, UK.

    Papers in Europe PMC
  10. 10
    Grosso F3 papers · 2026

    Mesothelioma, Melanoma and Rare Cancers Unit, Azienda Ospedaliera Universitaria, Alessandria, Italy.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

310

interventional trials for this specific condition

310 interventional trials matched this specific condition name; 46 currently recruiting in our sample. 158 trials are registered for mesothelioma, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026

310 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 99.6th percentile).

high confidence · 99.6th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

310 interventional trials matched after quoted-phrase search and title/condition post-filter.

Broader category: mesothelioma

158

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

Observational and natural-history studies

45 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.

Recruiting or not-yet-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 55 · after dedupe 54 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 54 · dropped 0 · fetched 2026-07-29

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).

Uncertain / not reviewed (54)

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 Pleural mesothelioma — 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.

"Pleural mesothelioma" OR "malignant mesothelial tumor" OR "malignant mesothelial tumour" OR "malignant mesothelioma" OR "malignant mesothelioma (disease)" OR "malignant neoplasm of mesothelium" OR "malignant neoplasm of the mesothelium" OR "malignant tumor of mesothelium" OR "malignant tumor of the mesothelium" OR "malignant tumour of mesothelium" OR "malignant tumour of the mesothelium" OR "mesothelioma, malignant" OR "mesothelioma, somatic"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: [OBSOLETE] Mesothelioma, Malignant

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Pleural mesothelioma" OR "malignant mesothelial tumor" OR "malignant mesothelial tumour" OR "malignant mesothelioma" OR "malignant mesothelioma (disease)" OR "malignant neoplasm of mesothelium" OR "malignant neoplasm of the mesothelium" OR "malignant tumor of mesothelium" OR "malignant tumor of the mesothelium" OR "malignant tumour of mesothelium" OR "malignant tumour of the mesothelium" OR "mesothelioma, malignant" OR "mesothelioma, somatic" OR "[OBSOLETE] Mesothelioma, Malignant"

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: 310 interventional · 45 observational · 2 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"mesothelioma"

Query health: suspect — strategies attempted: phrase, mesh; with hits: phrase

Run this search on ClinicalTrials.gov

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T00:18:41.986Z