Pleural mesothelioma (PMe): The evolving molecular knowledge of a rare and aggressive cancer

Author:

Rigon Manuel12,Mutti Luciano34,Campanella Michelangelo156ORCID

Affiliation:

1. Centre for Clinical Pharmacology and Precision Medicine William Harvey Research Institute Queen Mary University of London UK

2. Department of Biology University of Rome Tor Vergata Rome Italy

3. Department of Biotechnological and Applied Clinical Sciences DISCAB, L'Aquila University L'Aquila Italy

4. Temple University Sbarro Institute for Cancer Research and Molecular Medicine Philadelphia PA USA

5. Department of Biomedical Sciences University of Padua Padua Italy

6. Institute Gustave Roussy Villejuif France

Abstract

Mesothelioma is a type of late‐onset cancer that develops in cells covering the outer surface of organs. Although it can affect the peritoneum, heart, or testicles, it mainly targets the lining of the lungs, making pleural mesothelioma (PMe) the most common and widely studied mesothelioma type. PMe is caused by exposure to fibres of asbestos, which when inhaled leads to inflammation and scarring of the pleura. Despite the ban on asbestos by most Western countries, the incidence of PMe is on the rise, also facilitated by a lack of specific symptomatology and diagnostic methods. Therapeutic options are also limited to mainly palliative care, making this disease untreatable. Here we present an overview of biological aspects underlying PMe by listing genetic and molecular mechanisms behind its onset, aggressive nature, and fast‐paced progression. To this end, we report on the role of deubiquitinase BRCA1‐associated protein‐1 (BAP1), a tumour suppressor gene with a widely acknowledged role in the corrupted signalling and metabolism of PMe. This review aims to enhance our understanding of this devastating malignancy and propel efforts for its investigation.

Funder

H2020 European Research Council

Fondation ARC pour la Recherche sur le Cancer

Associazione Italiana per la Ricerca sul Cancro

Publisher

Wiley

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