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Mesothelioma

contributed by Dr Frank Gaillard on February 23, 2010

System: Chest
Tags: pleura
lungs

A mesothelioma in general is an aggressive malignant tumour (except for rare sub types such multicystic / cystic mesothelioma 8,9). The overwhelming majority arises from the pleura which this article will focus on. The reader is referred to a separate articles for discussion of 

Epidemiology

Mesotheliomas are uncommon entities and account for 5 - 28 % of all malignancies that involved the pleura1,7. There is a strong relationship between exposure to asbestos fibres and this tumour, and unlike other asbestos related lung disease, it doesn't appear to be dose dependant 1. Not all types of asbestos is strongly implicated, with crocidolite being the main causative fibre type. Not surprisingly, given the sources of asbestos exposure being predominantly mining, construction, lagging and machinery mechanics, 60 - 80% of cases are encountered in males, in general 20 to 35 years after exposure 1,5,6.

There has been no convincing evidence to suggest an association with smoking 6. 

Clinical presentation

Typically patients present with dyspnoea and low posterior non-pleuritic chest pain. Pleural effusions are seen in the vast majority of patients at some stage during their disease. Up to 25% of patients have metastatic disease at the time of presentation if staged with FDG PET 5. 

Pathology

There are three histological types of mesothelioma:

  1. epithelial : ~ 60% *
  2. mixed : 25%
  3. sarcomatoid : 15%

The cytological and histological diagnosis can be difficult, with mesothelial hyperplasia and metastatic adenocarcinoma appearing similar. Specific markers are helpful including:

  • calretinin
  • epithelial membrane antigen
  • cytokeratin
  • mesothelin (elevated in 84 % of malignant mesothelioma versus < 2 % with other pleural disease 6)

Radiographic features

See staging of malignant pleural mesothelioma.

Plain film

Chest x-rays are of limited utility and non specific 6 , demonstrating a pleural opacity which may extend around and encase the lung. Reduction in volume of the affected hemithorax is common resulting in shift of the mediastinum towards the lesion 4.

Rib destruction or extension beyond the lateral and anterior margins of the chest wall may be evident. Mediastinal lymph node enlargement may also be seen.

CT

CT is the most used modality for assessment of mesothelioma, and is able to stage the disease accurately in most patients.

The appearance is that of soft tissue attenuation nodular mass which spreads along pleural surfaces including into pleural fissures. 

Calcification is seen in 20% of cases which usually represents engulfed calcified pleural plaques rather than true tumour calcification 4. Sarcomatoid variants may demonstrate osteosarcoma or chondrosarcomatous components which may also be calcified.

An uncommon variant is the solitary mediastinal malignant mesothelioma which has appearances reminiscent of a solitary fibrous tumour of the pleura 1. 

Mesotheliomas have a predilection of direct invasion of adjacent structures (chest wall, diaphragm and mediastinal content) but also frequently metastasise to contralateral lung and local nodes1-2,4.

To confidently predict chest wall invasion the extrapleural fat plane should be seen to be infiltrated and / or direct extension in bone or muscle identified 4.

Presence of a pericardial effusion suggests transpericardial extension 3-4. 

MRI

MRI although not routinely used may have a role in refining the staging and better delineation in surgical candidates especially with regard to chest wall and diaphragmatic invasion 4.

  • T1 : iso to slightly hyperintense c.f muscle 4,6
  • T2 : iso to hyper intense c.f muscle 4,6
  • C+ (GAD) : enhancement usually present
PET

Positron emission tomography is becoming useful in two clinical settings 4:

  1. differentiating between benign and malignant asbestos related pleural thickening
  2. assessing for nodal metastases

In addition there appears to be a correlation between the degree of FDG uptake and the biological aggressiveness of the tumour, which may help guide treatment 4.

Treatment and prognosis

Treatment continues to be challenging and long term survival poor. Single modality treatment (surgery, radiotherapy, chemotherapy, immunotherapy and even photodynamic therapy) have not shown to improve survival 3. More recently multi-modality treatment has had some impact on favourable sub groups (early disease, and epithelioid histology). Treatment includes:

  1. extrapleural pneumonectomy
  2. adjuvant chemotherapy
  3. radiotherapy

Prognosis is poor for all tumour types with an overall medial survival without treatment of 4 - 12 months 3. In favourable patient sub-groups up to 45% 5 year survival may be achievable 3, however even with aggressive multi-modality therapy overall 5 year survival remains poor (3 - 18%) 3 with an median survival time of approximately 18 months 4.

Differential diagnoses