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An asbestos-caused cancer of the lung lining. Immunotherapy doublets replaced chemotherapy in 2020, and mesothelin CAR-T is under study.
Malignant pleural mesothelioma arises from the lining of the lung, almost always decades after asbestos exposure, and is rising in countries that banned asbestos late or not at all. It grows along surfaces rather than as a mass, is hard to image and stage, and resists most systemic therapy. Histology is the dominant biological variable: epithelioid tumours are slower and chemosensitive; sarcomatoid and biphasic tumours are aggressive, chemoresistant, and paradoxically more immunotherapy-responsive.
For 16 years after pemetrexed-cisplatin (2004) nothing improved survival except, modestly, adding bevacizumab (MAPS, 2016). Immunotherapy then changed the first line twice: nivolumab-ipilimumab (CheckMate 743, approved 2020) and pembrolizumab with chemotherapy (IND.227/KEYNOTE-483, approved September 2024). Tumour treating fields hold a device approval on single-arm data. Radical surgery, long assumed beneficial, was shown by MARS 2 (2024) to shorten survival and worsen quality of life, and is now largely confined to trials.
What comes next is biology-led: PRMT5 and MAT2A inhibitors for the ~40-50% of tumours with MTAP deletion, mesothelin-directed CAR-T delivered into the pleural space, ADCs and T-cell engagers against mesothelin, and better use of histology and BAP1/CDKN2A status to choose therapy. Prevention remains the biggest lever: asbestos is still mined and used in parts of Asia, Russia, and Brazil.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
Mesothelioma causes about 30,000 cases a year worldwide, ~3,000 in the US, almost all from asbestos exposure 20-50 years earlier, so incidence is still rising in Asia and parts of Europe. Median survival across series is 12-18 months, which means half of the people in those series lived longer.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
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.
Same organ: Mediastinal germ cell tumour, Pleuropulmonary blastoma (types I, Ir, II and III), Type A and type AB thymoma, Type B1 and type B2 thymoma, Type B3 thymoma, Micronodular thymoma with lymphoid stroma, Adenocarcinoma of the lung, Squamous cell carcinoma of the lung, Large cell carcinoma of the lung, Sarcomatoid carcinoma of the lung, Adenosquamous carcinoma of the lung, Invasive mucinous adenocarcinoma of the lung, Adenocarcinoma in situ and minimally invasive adenocarcinoma of the lung, Basaloid squamous cell carcinoma of the lung, Lymphoepithelial carcinoma of the lung, Pulmonary blastoma (adult), Non-small-cell lung cancer, Lung cancer (all types), Small-cell lung cancer, Pleural mesothelioma, Thymoma and thymic carcinoma, Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours), Inflammatory myofibroblastic tumour (IMT), EGFR-mutated non-small-cell lung cancer, ALK-positive non-small-cell lung cancer, KRAS G12C-mutant non-small-cell lung cancer, ROS1-positive non-small-cell lung cancer, MET exon 14 and MET-amplified non-small-cell lung cancer, RET fusion-positive non-small-cell lung cancer, BRAF V600E-mutant non-small-cell lung cancer, HER2-mutant non-small-cell lung cancer, NTRK fusion-positive non-small-cell lung cancer, PD-L1-high non-small-cell lung cancer without a driver mutation, Resectable stage I to III non-small-cell lung cancer, Unresectable stage III non-small-cell lung cancer, Limited-stage small-cell lung cancer, Extensive-stage small-cell lung cancer, Lung neuroendocrine tumours (typical and atypical carcinoid), Large cell neuroendocrine carcinoma of the lung, Thymoma (WHO types A, AB, B1, B2 and B3), Thymic carcinoma
Most people reading this do not have advanced disease. The map describes what can happen over the whole course of the illness, across autopsy and registry series; today's staging scans find spread earlier, and each site has treatments, from focused radiotherapy for a few spots to drugs that reach the brain.
What helpsNivolumab-ipilimumab or pembrolizumab with chemotherapy first line; mesothelin CAR-T delivered into the pleural space and PRMT5 inhibitors for MTAP-deleted tumours are in trials.
Also on OnCo: Atlas of advanced disease · How cancer spreads: the metastasis stages.
Grows along the pleura and into the chest wall and diaphragm; distant spread is late.
Nivolumab-ipilimumab or chemo-IO; TTFields.
CT and PET/CT; thoracoscopic biopsy with IHC panel (calretinin, WT1, D2-40; BAP1 and MTAP loss support malignancy); histology and BAP1/MTAP status recorded for treatment planning.
Nivolumab + ipilimumab (CheckMate 743) preferred; pembrolizumab + platinum-pemetrexed alternative.
Pembrolizumab + platinum-pemetrexed, nivolumab + ipilimumab, or platinum-pemetrexed ± bevacizumab, chosen by fitness and preference.
Continue immunotherapy per regimen; TTFields (Optune Lua) with pemetrexed-platinum under HDE approval; no maintenance chemotherapy standard.
Nivolumab (CONFIRM: OS benefit vs placebo) or nivolumab-ipilimumab if not given first line; platinum-pemetrexed rechallenge or gemcitabine/vinorelbine if IO given first; trials.
Not recommended for cure outside trials after MARS 2; VATS pleurodesis or indwelling pleural catheter for effusion; extended pleurectomy/decortication (P/D) only in clinical trials.
Palliative for chest wall pain; prophylactic tract irradiation not beneficial (SMART/PIT trials); hemithoracic IMRT after surgery only in trials.
Cytoreductive surgery with HIPEC in selected patients; systemic therapy extrapolated from pleural disease.
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.
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.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
No pharmacokinetic interactions expected (antibody). See the irAE guide for toxicity management.
Not recommended for CrCl below 45.
Immunotherapy can attack hormone-producing glands: most often the thyroid (usually ending in an under-active thyroid needing lifelong tablets), and less often the pituitary (hypophysitis) or adrenal glands, which can be life-threatening if missed.
Inflammation of the bowel caused by immunotherapy releasing the immune system against the gut lining, producing diarrhoea that can be severe. It is the commonest serious side effect of CTLA-4 antibodies and is treated with steroids and, if needed, infliximab.
See all on the product pages:IpilimumabNivolumabPembrolizumabPemetrexed·Printable cards in the navigator
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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.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.