Rare thoracic cancers: a comprehensive overview of diagnosis and management of small cell lung cancer, malignant pleural mesothelioma and thymic epithelial tumours

Our plain-English summary of Rare thoracic cancers: a comprehensive overview of diagnosis and management of small cell lung cancer, malignant pleural mesothelioma and thymic epithelial tumours, by D.W. Dumoulin, P. Bironzo, F. Passiglia, et al (European Respiratory Review, 2023).

The study

• Authors: D.W. Dumoulin, P. Bironzo, F. Passiglia, G.V. Scagliotti, J.G.J.V. Aerts, ERN-LUNG Core Network Mesothelioma
• Journal: European Respiratory Review
• Published: 2023;32(167)
• Type of article: Review
• Cited by: 11 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Despite the progress in outcomes seen with immunotherapy in various malignancies, including nonsmall cell lung cancer, the benefits are less in small cell lung cancer, malignant pleural mesothelioma and thymic epithelial tumours. New effective treatment options are needed, guided via more in-depth insights into the pathophysiology of these rare malignancies. This review comprehensively presents an overview of the clinical presentation, diagnostic tools, staging systems, pathophysiology and treatment options for these rare thoracic cancers. In addition, opportunities for further improvement of therapies are discussed.

Copyright ©The authors 2023.

In plain English

This article was a commissioned, peer-reviewed review, not an original study. The authors summarised published research and guidelines on three rare chest cancers: small cell lung cancer, malignant pleural mesothelioma and thymic epithelial tumours. In the mesothelioma section, they described mesothelioma as classified by the WHO as directly related to all types of asbestos exposure. They reported that 30 000 people were diagnosed worldwide in 2020, with an incidence of 0.7 per 100 000 in males and 0.3 per 100 000 in females. They wrote that people who washed the clothes of asbestos workers are also at risk, and that the time from exposure to onset is generally more than 30 years. They noted that a cited meta-analysis urged caution about some risk estimates for non-occupational exposure. Citing other studies, they reported a median survival of 9 months without therapy. From the CheckMate 743 trial, they reported 3-year survival of 23.2% with nivolumab plus ipilimumab versus 15.4% with chemotherapy. The authors concluded that new effective treatments are needed. They also concluded that, since 2020, nivolumab plus ipilimumab is to be considered standard first-line therapy for mesothelioma.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Narrative review (commissioned, peer-reviewed article in a series on rare lung diseases); no new patient data
• Who was studied: No study population. The review covered published literature on three rare thoracic cancers: small cell lung cancer, malignant pleural mesothelioma and thymic epithelial tumours
• Exposure: For mesothelioma, the review discussed asbestos exposure in general ("all types of asbestos"): occupational exposure, exposure from washing a worker's clothes, and neighbourhood, domestic and household exposure. No specific fiber types were named. For small cell lung cancer, the exposure discussed was tobacco smoking.
• Main results:
  • Citing other sources, the review reported that 30 000 patients worldwide were diagnosed with mesothelioma in 2020, with a higher incidence in males (0.7 per 100 000) than in females (0.3 per 100 000), increasing with age
  • The review stated that individuals who washed the clothes of someone who worked with asbestos are also at risk of mesothelioma
  • The review stated that non-occupational exposure (neighbourhood, domestic and household) is associated with an increased risk of mesothelioma. It added that a cited meta-analysis indicated some summary relative risk estimates should be interpreted with caution because of high between-studies heterogeneity.
  • The review stated that the time between asbestos exposure and onset of mesothelioma is in general more than 30 years
  • Citing other work, the review stated that the risk of malignant transformation is higher when asbestos fibres are longer than 10 micrometres, because macrophages clear them less easily
  • Citing an earlier trial, the review reported a median survival of only 9 months for mesothelioma without therapy
  • Citing the phase III CheckMate 743 trial, the review reported 3-year overall survival of 23.2% with nivolumab plus ipilimumab versus 15.4% with standard chemotherapy
  • Citing the MARS study, the review reported that extrapleural pneumonectomy failed to show a benefit when added to standard chemotherapy
  • The review stated that 50 to 70% of pleural and peritoneal mesotheliomas show BAP1 loss
  • The review stated that there is currently no indication for a systematic early detection programme in the asbestos-exposed population
• The authors' conclusion: The authors concluded that new effective treatments are needed for these rare cancers. For mesothelioma, they concluded that it arises from exposure to asbestos fibres through an inflammatory reaction. They also concluded that, since 2020, nivolumab plus ipilimumab is to be considered standard first-line therapy regardless of histological subtype.

Topics

The National Library of Medicine indexes this study under subjects our site covers: Lung cancer, Mesothelioma.

Medical terms in this study, explained in our Lexicon: Small Cell Lung Cancer, Pleural Mesothelioma, Immunotherapy, Lung Cancer, Staging.

Related studies

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• Primary thymic epithelial tumours of the pleura mimicking malignant mesothelioma — R.L. Attanoos, F. Galateau-Salle, et al. (2002)
• Small cell mesothelioma: A rare entity and diagnostic pitfall mimicking small cell lung carcinoma on fine-needle aspiration — Y. Zhang, A. Afify, et al. (2016)
• Factors influencing exercise performance in thoracic cancer — R. England, M. Maddocks, et al. (2012)
• Utility of PAX8 mouse monoclonal antibody in the diagnosis of thyroid, thymic, pleural and lung tumours: a comparison with polyclonal PAX8 antibody — A. Toriyama, T. Mori, et al. (2014)
• Loss of BRCA1-associated protein 1 (BAP1) expression is rare in non-small cell lung cancer — D. Owen, B.S. Sheffield, et al. (2017)

Browse more research in European Respiratory Review, or return to medical research.