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Malignant mesothelioma is a fatal asbestos-associated malignancy arising in the lining cells (mesothelium) of the pleural and peritoneal cavities, as well as in the pericardium and the tunica vaginalis.
ORPHA:50251Classification level: Disorder
- Synonym(s): -
- Prevalence: 1-9 / 100 000
- Inheritance: Not applicable
- Age of onset: All ages
- ICD-10: C45.0
- OMIM: 156240
- UMLS: C0025500 C0812413 C1377913
- MeSH: D008654
- GARD: -
- MedDRA: 10027406
The exact prevalence is unknown but it is estimated that mesotheliomas represent less than 1% of all cancers. Its incidence is increasing, with an expected peak in the next 10-20 years.
Pleural malignant mesothelioma is the most common form of mesothelioma. Typical presenting features are chest pain and dyspnea. Breathlessness due to a pleural effusion without chest pain is reported in about 30% of patients. A chest wall mass, weight loss, sweating, abdominal pain and ascites (due to peritoneal involvement) are less common presentations.
Mesothelioma is directly attributable to occupational asbestos exposure with a history of exposure in over 90% of cases. There is also evidence that mesothelioma may result from both para-occupational exposure and non-occupational 'environmental' exposure. Idiopathic or spontaneous mesothelioma can also occur in the absence of any exposure to asbestos, with a spontaneous rate in humans of around one per million.
A combination of accurate exposure history, along with examination radiology and pathology are essential to make the diagnosis. The most suggestive CT findings indicating malignant pleural disease are 1) a circumferential pleural rind, 2) nodular pleural thickening, 3) pleural thickening of >1cm and 4) mediastinal pleural involvement.
Distinguishing malignant from benign pleural disease can be challenging.
Management and treatment
Involvement of a multidisciplinary team is recommended to ensure prompt and appropriate management, using a framework of radiotherapy, chemotherapy, surgery and symptom palliation with terminal care. Compensation issues must also be considered.
Life expectancy in malignant mesothelioma is poor, with a median survival of about one year following diagnosis.