# Pleural mesothelioma

Source: https://onco.cc/cancers/pleural-mesothelioma/  
OnCo record `pleural-mesothelioma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Pleural mesothelioma grows in the lining of the lung after asbestos exposure and causes breathlessness and chest pain from fluid and thickening. It is rarely curable; the first-line choice is nivolumab with ipilimumab or pembrolizumab with chemotherapy, and surgery to remove the lining is no longer recommended outside trials.

## Summary

Pleural mesothelioma is the commonest form of mesothelioma. It presents with pleural effusion, breathlessness and chest wall pain 20 to 50 years after asbestos exposure, and is diagnosed by thoracoscopic biopsy with immunohistochemistry (calretinin, WT1, D2-40) and loss of BAP1 or MTAP. Epithelioid histology, about two thirds of cases, carries a better outlook than biphasic or sarcomatoid disease. CheckMate 743 made nivolumab plus ipilimumab the first new first-line treatment in 16 years, and KEYNOTE-483 added pembrolizumab with platinum-pemetrexed; both are now standard, with histology guiding the choice. MARS 2 showed that extended pleurectomy and decortication harms rather than helps, so surgery is confined to diagnosis, pleurodesis and trials. Hemithoracic radiotherapy, tumour treating fields and mesothelin-directed therapies are the main lines of research.

## Fields

- Kind: Cancer
- Last checked: 2026-09-16
- Also known as: Malignant pleural mesothelioma; MPM
- Tags: subtype-page
- Group: thoracic
- Burden: About four in five mesotheliomas arise in the pleura; roughly 2,500 cases a year in the UK and 3,000 in the US, almost all decades after asbestos exposure, with incidence still rising in countries that banned asbestos late.
- Subtypes: Epithelioid (about two thirds); Biphasic; Sarcomatoid (including desmoplastic)
- Biomarkers: Histology (epithelioid versus non-epithelioid); BAP1 loss; CDKN2A/MTAP deletion; Mesothelin expression; PD-L1 (not predictive for nivolumab plus ipilimumab)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/pleural-mesothelioma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/pleural-mesothelioma/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/pleural-mesothelioma/#what-it-is [3 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/pleural-mesothelioma/#finding-it [5 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/pleural-mesothelioma/#treating-it [5 settings, 2 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/pleural-mesothelioma/#evidence [9 trials, 4 key papers, 5 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/pleural-mesothelioma/#science [19 targets]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/pleural-mesothelioma/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/pleural-mesothelioma/#living-with-it [20 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/pleural-mesothelioma/coming/ [5 medicines, 9 trials, 1 idea, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/pleural-mesothelioma/data/ [50 connected records]

## Standard of care

- Diagnosis and staging: CT and PET-CT; thoracoscopic biopsy with an immunohistochemistry panel; histology and BAP1/MTAP status recorded because they drive treatment. ([Mesothelioma](https://onco.cc/cancers/mesothelioma/))
- First line, non-epithelioid: Nivolumab plus ipilimumab (CheckMate 743). ([Nivolumab](https://onco.cc/drugs/nivolumab/), [Ipilimumab](https://onco.cc/drugs/ipilimumab/), [CheckMate 743](https://onco.cc/trials/checkmate-743/))
- First line, epithelioid: Pembrolizumab with platinum-pemetrexed (KEYNOTE-483), nivolumab plus ipilimumab, or platinum-pemetrexed with or without bevacizumab, by fitness and preference. ([Pembrolizumab](https://onco.cc/drugs/pembrolizumab/), [Pemetrexed](https://onco.cc/drugs/pemetrexed/), [IND.227 / KEYNOTE-483](https://onco.cc/trials/keynote-483/))
- Surgery: Not recommended for cure outside trials after MARS 2; talc pleurodesis or an indwelling pleural catheter controls effusion. ([MARS 2](https://onco.cc/trials/mars-2/))
- Second line: Nivolumab (CONFIRM) if not given first line; chemotherapy rechallenge or gemcitabine or vinorelbine after immunotherapy. ([Nivolumab](https://onco.cc/drugs/nivolumab/))

## State of the art

- Immunotherapy doubled the share of patients alive at five years compared with chemotherapy alone, but most patients still die of the disease within two years.
- Randomised evidence has removed radical surgery from standard care, sparing patients a major operation with no benefit.
- Mesothelin is expressed on almost every tumour cell, and the next generation of antibody-drug conjugates and CAR-T cells is built on it.

## Open problems

- No curative treatment for the majority; median survival with the best regimens is about 18 months.
- Sarcomatoid disease responds poorly to everything except immunotherapy.
- No screening test for exposed workers, though BAP1 germline carriers and asbestos cohorts are being followed.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Mesothelioma
- Wikipedia: https://en.wikipedia.org/wiki/Mesothelioma
- Mesothelioma UK: https://www.mesothelioma.uk.com/

## Connected records

- cancers: [Mesothelioma](https://onco.cc/cancers/mesothelioma/), [Peritoneal mesothelioma](https://onco.cc/cancers/peritoneal-mesothelioma/)
- terms: [Asbestos](https://onco.cc/terms/asbestos/), [BAP1 loss](https://onco.cc/terms/bap1-loss/), [CDKN2A/B homozygous deletion](https://onco.cc/terms/cdkn2a-homozygous-deletion/), [Malignant pleural effusion](https://onco.cc/terms/malignant-pleural-effusion/)
- key papers: [CheckMate 743: first-line nivolumab plus ipilimumab in unresectable pleural mesothelioma](https://onco.cc/key-papers/paper-checkmate-743-lancet-2021/), [KEYNOTE-483 (CCTG IND.227): pembrolizumab plus chemotherapy versus chemotherapy in untreated pleural mesothelioma](https://onco.cc/key-papers/paper-keynote-483-lancet-2023/), [MARS 2: extended pleurectomy decortication plus chemotherapy versus chemotherapy alone for pleural mesothelioma](https://onco.cc/key-papers/paper-mars-2-lancet-respir-med-2024/), [Phase 3 trial of pemetrexed plus cisplatin versus cisplatin alone in malignant pleural mesothelioma](https://onco.cc/key-papers/paper-vogelzang-pemetrexed-mesothelioma-jco-2003/)
- drugs: [Anetumab ravtansine](https://onco.cc/drugs/anetumab-ravtansine/), [Ipilimumab](https://onco.cc/drugs/ipilimumab/), [Nivolumab](https://onco.cc/drugs/nivolumab/), [Pembrolizumab](https://onco.cc/drugs/pembrolizumab/), [Pemetrexed](https://onco.cc/drugs/pemetrexed/)
- trials: [AMPLE](https://onco.cc/trials/ample/), [CheckMate 743](https://onco.cc/trials/checkmate-743/), [Dual-Target MSLN/FAP CAR-NK Cells for Pleural and Peritoneal Mesothelioma](https://onco.cc/trials/nct07510815/), [IND.227 / KEYNOTE-483](https://onco.cc/trials/keynote-483/), [MARS 2](https://onco.cc/trials/mars-2/), [MEDI5752 in Combination With Carboplatin Plus Pemetrexed in Unresectable Pleural Mesothelioma](https://onco.cc/trials/nct06097728/), [Randomized, Double-blind Study Comparing Tremelimumab to Placebo in Subjects With Unresectable Malignant Mesothelioma](https://onco.cc/trials/nct01843374/), [TIME2](https://onco.cc/trials/time2/), [TT-4 and TT-10 as Single Agents and in Combination in Subjects With Advanced Selected Solid Tumors and Pleural Mesothelioma](https://onco.cc/trials/nct04969315/)
- ideas: [Antibody-drug conjugates for mesothelioma: why they have failed so far and how they could work](https://onco.cc/ideas/idea-adc-for-mesothelioma/)
- people: [Quincy Chu](https://onco.cc/people/quincy-chu/)
- targets: [ARHGAP5](https://onco.cc/targets/arhgap5/), [CYP2C8](https://onco.cc/targets/cyp2c8/), [DGCR8](https://onco.cc/targets/dgcr8/), [FLG](https://onco.cc/targets/flg/), [HLA-DQA1](https://onco.cc/targets/hla-dqa1/), [LATS2](https://onco.cc/targets/lats2/), [MGA](https://onco.cc/targets/mga/), [NFKB2](https://onco.cc/targets/nfkb2/), [NIN](https://onco.cc/targets/nin/), [PDZD2](https://onco.cc/targets/pdzd2/), [PI4KA](https://onco.cc/targets/pi4ka/), [SHTN1](https://onco.cc/targets/shtn1/), [SRGAP3](https://onco.cc/targets/srgap3/), [TAF15](https://onco.cc/targets/taf15/), [TRAF7](https://onco.cc/targets/traf7/), [WNK2](https://onco.cc/targets/wnk2/)

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JSON: https://onco.cc/api/v1/entities/pleural-mesothelioma.json