Pleural mesothelioma
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.
Overview
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.
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.
Anatomy and lymph node drainage
Central tumours arise in the large airways, peripheral ones in the alveoli; both drain to hilar then mediastinal nodes, and the pleural lining is a separate cancer site.
- Central airways (squamous, small-cell)
- Periphery (adenocarcinoma)
- Apex (Pancoast)
- Pleura (mesothelioma)Epithelioid (about two thirds) · Biphasic · Sarcomatoid (including desmoplastic)
- Thymus (anterior mediastinum)
- hilar (N1)
- mediastinal (N2)
- supraclavicular (N3)
Same organ: Non-small-cell lung cancer, Lung cancer (all types), Small-cell lung cancer, Mesothelioma, Thymoma and thymic carcinoma, Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours), Inflammatory myofibroblastic tumour (IMT)
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.
- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
CT and PET-CT; thoracoscopic biopsy with an immunohistochemistry panel; histology and BAP1/MTAP status recorded because they drive treatment.
Nivolumab plus ipilimumab (CheckMate 743).
Pembrolizumab with platinum-pemetrexed (KEYNOTE-483), nivolumab plus ipilimumab, or platinum-pemetrexed with or without bevacizumab, by fitness and preference.
Not recommended for cure outside trials after MARS 2; talc pleurodesis or an indwelling pleural catheter controls effusion.
Nivolumab (CONFIRM) if not given first line; chemotherapy rechallenge or gemcitabine or vinorelbine after immunotherapy.
Subtypes & biomarkers
top- Epithelioid (about two thirds)
- Biphasic
- Sarcomatoid (including desmoplastic)
- Histology (epithelioid versus non-epithelioid)
- BAP1 loss
- CDKN2A/MTAP deletion
- Mesothelin expression
- PD-L1 (not predictive for nivolumab plus ipilimumab)
How often this target appears
- 1960Wagner links pleural mesothelioma to asbestos
Thirty-three cases among asbestos miners and their neighbours in South Africa.
- 2003Pemetrexed-cisplatin improves survival
- 2020CheckMate 743: first immunotherapy survival gain
- 2024MARS 2 ends radical surgery as standard
- 2024Pembrolizumab with chemotherapy approved
What is in development for Pleural mesothelioma, drawn from the whole corpus: 1 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Ideas not yet in a trial · 1
Open problems and what is being done
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.
and how the field plans to fix it →What is being done about thisFinding cancer earlierAvailable now- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
In trialsIdeas and roadmaps- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Trials
topTrials recruiting now
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Landmark trials
Expert centres
topExpert centres
- via Mesothelioma
- via Mesothelioma
- Johns Hopkins Hospital / Sidney Kimmel Comprehensive Cancer CenterBaltimore, USNewsweek oncology #10NCI comprehensivevia Pembrolizumab
- via Mesothelioma
- via Mesothelioma
- via Nivolumab, Ipilimumab
- via Nivolumab, Ipilimumab
- via Nivolumab, Ipilimumab
- via Nivolumab
- Alliance for Clinical Trials in OncologyChicago, IL, USvia Pembrolizumab
- ETOP IBCSG Partners FoundationBern, CHvia Mesothelioma
- German Hodgkin Study GroupCologne, DEvia Nivolumab
- GOG FoundationPhiladelphia, PA, USvia Pembrolizumab
- Institut Jules BordetBrussels, BEvia Pembrolizumab
- via Mesothelioma
- International Association for the Study of Lung CancerDenver, CO, USvia Mesothelioma
- Kyoto University HospitalKyoto, JPvia Nivolumab
- via Nivolumab
- via Pembrolizumab
- via Mesothelioma
- National Cancer Center Hospital EastKashiwa, Chiba, JPvia Nivolumab
- NCI Center for Cancer Research (intramural programme)Bethesda, MD, USvia Mesothelioma
- Shanghai Chest HospitalShanghai, CNvia Mesothelioma
- SWOG Cancer Research NetworkPortland, OR, USvia Nivolumab
Questions to ask
topQuestions to ask your oncologist about Pleural mesothelioma
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Histology, BAP1 loss, CDKN2A/MTAP deletion, Mesothelin expression, PD-L1), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Epithelioid, Biphasic, Sarcomatoid.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Diagnosis and staging
- For my situation (diagnosis and staging), which of the standard options do you recommend and why?Why: Guideline options include: CT and PET-CT; thoracoscopic biopsy with an immunohistochemistry panel; histology and BAP1/MTAP status recorded because they drive treatment.
First line, non-epithelioid
- For my situation (first line, non-epithelioid), which of the standard options do you recommend and why?Why: Guideline options include: Nivolumab plus ipilimumab (CheckMate 743).
- Am I a candidate for Nivolumab, Ipilimumab, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of CheckMate 743 apply to someone like me?Why: Trial populations differ from individual patients; ask how closely you match.
First line, epithelioid
- For my situation (first line, epithelioid), which of the standard options do you recommend and why?Why: Guideline options include: Pembrolizumab with platinum-pemetrexed (KEYNOTE-483), nivolumab plus ipilimumab, or platinum-pemetrexed with or without bevacizumab, by fitness and preference.
- Am I a candidate for Pembrolizumab, Pemetrexed, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of IND.227 / KEYNOTE-483 apply to someone like me?Why: Trial populations differ from individual patients; ask how closely you match.
Surgery
- For my situation (surgery), which of the standard options do you recommend and why?Why: Guideline options include: Not recommended for cure outside trials after MARS 2; talc pleurodesis or an indwelling pleural catheter controls effusion.
- How do the results of MARS 2 apply to someone like me?Why: Trial populations differ from individual patients; ask how closely you match.
Second line
- For my situation (second line), which of the standard options do you recommend and why?Why: Guideline options include: Nivolumab (CONFIRM) if not given first line; chemotherapy rechallenge or gemcitabine or vinorelbine after immunotherapy.
- Am I a candidate for Nivolumab, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Antibody-drug conjugates for mesothelioma: why they have failed so far and how they could work?Why: Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “No curative treatment for the majority; median survival with the best regimens is about 18 months”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Sarcomatoid disease responds poorly to everything except immunotherapy”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
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Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
2targets
2drugs
4companies
2trials
3ideas
1Latest papers
topQuery for this cancer: (TITLE:"Pleural mesothelioma" OR ABSTRACT:"Pleural mesothelioma" OR TITLE:"Malignant pleural mesothelioma" OR ABSTRACT:"Malignant pleural mesothelioma" OR TITLE:"MPM" OR ABSTRACT:"MPM") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Pleural mesothelioma, not a curated reading list.
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