Disease Directory Malignant Pleural Mesothelioma
Oncology

Malignant Pleural Mesothelioma

Also known as: MPM, asbestos-related mesothelioma, peritoneal mesothelioma, BAP1 tumor

Prevalence

1–2 in 100,000

Onset

Adult (median age 70s; long latency of 30–50 years from asbestos exposure)

Type

Sporadic (asbestos-related); BAP1 tumour predisposition syndrome in familial cases

Gene

BAP1, NF2

About Malignant Pleural Mesothelioma

Malignant pleural mesothelioma is an aggressive, diffuse malignancy of the pleural mesothelium most strongly causally associated with prior asbestos exposure, with a latency period of 20–50 years. Loss-of-function mutations in BAP1 (BRCA1-associated protein 1), a nuclear deubiquitinase and tumour suppressor, and NF2 (merlin) are the most common somatic genetic alterations, with germline BAP1 mutations conferring the BAP1 tumour predisposition syndrome. The combination of nivolumab and ipilimumab (dual checkpoint blockade) has become a new first-line standard in non-epithelioid histology, but outcomes remain poor with median overall survival of 12–18 months for all comers.

Common Clinical Features

Progressive dyspnoea from pleural effusion or direct pleural encasement of the lung Chest pain: dull, constant, often pleuritic, worsening with inspiration Unexplained weight loss, cachexia, and fatigue Restricted chest expansion and decreased breath sounds on the affected side Phrenic nerve palsy causing paradoxical diaphragmatic movement Superior vena cava obstruction in advanced disease with mediastinal involvement Peritoneal extension causing ascites and abdominal distension in advanced cases

Clinical Trial Eligibility Tips

What to know before applying to Malignant Pleural Mesothelioma trials.

Histological subtype (epithelioid, sarcomatoid, or biphasic) must be confirmed on adequate tissue, as it is a primary eligibility and stratification criterion — fine needle aspirate alone is typically insufficient for trial entry.

Asbestos exposure history (occupational, domestic, or environmental) and documentation of latency period are required for certain aetiologically defined trials and for compensation-linked registry enrolment.

BAP1 IHC loss or germline BAP1 testing is increasingly requested for biomarker-selected trials and for BAP1 tumour predisposition syndrome family screening; arrange germline testing if tumour shows BAP1 loss.

Patient Resources

Patient Organization

Mesothelioma Applied Research Foundation

Visit website ↗

Natural History Registry

MARF Patient Registry

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Orphanet

European reference resource for rare diseases (ORPHA:50251)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting Malignant Pleural Mesothelioma trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Malignant Pleural Mesothelioma. Updated daily.

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