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Pleural tumours: mesothelioma and solitary fibrous tumour

  • Lesion

Mesothelioma is an aggressive cancer of the pleural lining caused mostly by asbestos. Solitary fibrous tumour is a usually benign pleural mass.

Updated

What it is

Most tumours in the pleura are metastases, especially from lung and breast. The two primary tumours to know are malignant mesothelioma, a diffuse asbestos-related cancer with a latency of decades and a poor prognosis, and solitary fibrous tumour, a localised fibroblastic tumour defined by a NAB2::STAT6 fusion and unrelated to asbestos. Immunohistochemistry separates mesothelioma from adenocarcinoma.

Structure

  • Mesothelioma gross: thick, soft, gelatinous grey-pink rind that encases the lung and fills the fissures, with effusion.
  • Epithelioid: cuboidal or flattened cells forming tubules and papillae, like adenocarcinoma.
  • Sarcomatoid: malignant spindle cells like a sarcoma; biphasic: both.
  • Solitary fibrous tumour: firm, well-circumscribed mass on a pedicle; patternless spindle cells among collagen and branching staghorn vessels.

Step by step

  1. Inhaled asbestos fibres (especially amphiboles) migrate to the pleura.
  2. Fibres cause chronic inflammation, oxidant injury and DNA damage in mesothelial cells.
  3. Over decades the cells lose CDKN2A, BAP1 and NF2 tumour suppressors.
  4. The tumour spreads over the pleural surfaces and invades the chest wall, lung and mediastinum.
  5. Solitary fibrous tumour arises from a NAB2::STAT6 fusion that creates a chimeric transcription factor.

Mesothelioma vs lung adenocarcinoma in the pleura

FeatureEpithelioid mesotheliomaAdenocarcinoma
GrowthDiffuse rind encasing the lungMass in the lung, pleural nodules
Positive markersCalretinin, WT1, CK5/6, D2-40TTF-1, napsin A, claudin-4, BerEP4, CEA
Main causeAsbestosTobacco
Smoking effectNo added riskStrong risk factor

Markers

  • Calretinin
  • WT1
  • CK5/6
  • D2-40 (podoplanin)
  • BAP1 loss
  • STAT6
  • CD34

Clinical notes

  • Mesothelioma: chest pain, dyspnoea and recurrent effusions; asbestosis is present in only a minority.
  • Median survival is about a year; peritoneal, pericardial and tunica vaginalis mesotheliomas also occur.
  • Solitary fibrous tumour can cause hypoglycaemia (IGF-2) or clubbing; resection is usually curative.

Test yourself

Think of the answer, then tap the question to see it.

Which immunohistochemical markers support mesothelioma over adenocarcinoma?

Calretinin, WT1, CK5/6 and podoplanin positive, with claudin-4, BerEP4 and TTF-1 negative.

What is the latency between asbestos exposure and mesothelioma?

Usually decades, about 25 to 45 years.

What fusion gene defines solitary fibrous tumour?

NAB2::STAT6, shown by nuclear STAT6 staining.

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Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.

Sources: Mesothelioma (MedlinePlus, US National Library of Medicine, 2025); Pleural Disorders (MedlinePlus, US National Library of Medicine, 2025).

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