Rare thoracic cancers - a comprehensive overview of diagnosis and management of small cell lung cancer, malignant pleural mesothelioma and thymic epithelial tumours - Series Number 3

Sep 24, 2026

Despite advances in immunotherapy outcomes across many malignancies, clinical benefit is more limited for small cell lung cancer, malignant pleural mesothelioma, and thymic epithelial tumours. Progress requires deeper mechanistic understanding of these rare diseases and the translation of that knowledge into effective therapies. This review provides a comprehensive overview of clinical presentation, diagnostic approaches, staging systems, underlying pathophysiology, and available treatment options, and discusses opportunities to further improve future management.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages16

Reading Time40 min

AccessFree to download

Shareable abstract

Review overview

Introduction

Clinical burden and treatment limitations

Small cell lung cancer

Classification and epidemiology

Etiology and risk factors

Diagnosis and histology

Immunohistochemistry and biomarkers

EUROPEAN RESPIRATORY REVIEW SERIES D.W. DUMOULIN ET AL. Rare thoracic cancers: a comprehensive overview of diagnosis and management of small cell lung cancer, malignant pleural mesothelioma and thymic epithelial tumours Daphne W. Dumoulin1, Paolo Bironzo2, Francesco Passiglia2, Giorgio V. Scagliotti 2 and Joachim G.J.V. Aerts1 on behalf of the ERN-LUNG Core Network Mesothelioma Number 3 in the Series “The world of rare lung diseases” Edited by Michael Kreuter, Marc Humbert, Thomas Wagner and Marlies Wijsenbeek 1 Department of Pulmonary Medicine, Erasmus MC Cancer Institute, Rotterdam, The Netherlands. 2 Department of Oncology, University of Torino, San Luigi Gonzaga Hospital, Orbassano, Italy. Corresponding author: Daphne W. Dumoulin ([d.dumoulin@erasmusmc.nl](d.dumoulin@erasmusmc.nl) ) Shareable abstract (@ERSpublications) SCLC, mesothelioma and thymoma are rare thoracic cancers. Diagnosis and treatment options should therefore be discussed in a multidisciplinary discussion. Although progress has been made with the entrance of ICI, novel strategies are being studied. [https://bit.ly/3TTUgY9](https://bit.ly/3TTUgY9) [Cite this article as:](Cite this article as: Dumoulin DW)[ Dumoulin DW](Cite this article as: Dumoulin DW), [Bironzo P](Bironzo P), [Passiglia F](Passiglia F), et al. Rare thoracic cancers: a comprehensive overview of diagnosis and management of small cell lung cancer, malignant pleural mesothelioma and thymic epithelial tumours. Eur Respir Rev 2023; 32: 220174 [DOI: 10.1183/16000617.0174-2022] . Copyright ©The authors 2023 This version is distributed under the terms of the Creative Commons Attribution NonCommercial Licence 4 .0. For commercial reproduction rights and permissions contact [permissions@ersnet.org](permissions@ersnet.org) This article has an editorial commentary: [https://doi.org/10.1183/](https://doi.org/10.1183/)[ ](https://doi.org/10.1183/)[16000617.0006-2023](16000617.0006-2023) Received: 30 Aug 2022 Accepted: 18 Oct 2022 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.
Introduction | | --- | | Small cell lung cancer (SCLC), malignant pleural mesothelioma (MPM) and thymic epithelial tumours (TETs) are rare but aggressive thoracic malignancies. The prognosis of patients is frequently dismal and treatment options are limited. Despite the progress in outcomes seen with immunotherapy in various malignancies, including nonsmall cell lung cancer (NSCLC), the benefits in these rare thoracic malignancies are less for partially unknown reasons. New effective treatment options are needed, guided via more in-depth insights into the pathophysiology of these rare malignancies, as we have learned from recently performed studies that general principles established in other malignancies do not apply to these rare cancers.
In this review, we provide an overview of the current landscape of these rare thoracic cancers. Based on completed and ongoing clinical trials, we will suggest therapeutic options for further improvement of therapies in the (near) future.
Small cell lung cancer | According to the 2021 World Health Organization (WHO) classification of lung tumours, SCLC is part of theneuroendocrine lung neoplasms, along with large cell neuroendocrine carcinomas and carcinoid tumours [1]. Epidemiology SCLC represents about 15% of all lung cancers. With an incidence of 1–5 per 10 000, SCL

FAQs

Why should diagnosis and treatment for SCLC, mesothelioma, and thymoma be discussed in a multidisciplinary setting?

These are rare thoracic cancers with limited treatment options, so care benefits from multidisciplinary discussion. The review emphasizes the need for coordinated diagnostic and therapeutic planning.

What factors limit the benefits of immunotherapy in small cell lung cancer and related rare thoracic malignancies?

The abstract states that, despite progress in immunotherapy for other malignancies, benefits are less in SCLC, malignant pleural mesothelioma, and thymic epithelial tumours. It attributes this to less favorable outcomes and partially unknown reasons.

How is the diagnosis of small cell lung cancer primarily established?

Diagnosis is primarily based on histological appearance on light microscopy. It shows small tumour cells, poorly defined cell borders, scant cytoplasm, and nuclear moulding, with a high mitotic rate.

Sep 24, 2026

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