Pleural mesothelioma
This reference system, the use of which is based on the ethical principles of personal practice of medicine, was drawn up by a multidisciplinary working group of professionals from the regional cancer research networks of Alsace (CAROL), Burgundy ( ONCOBOURGOGNE), Champagne-Ardenne (ONCOCHA), Franche-Comté (ONCOLIE), Lorraine (ONCOLOR) taking into account national recommendations and regulations, the Auvergne-Rhône-Alpes thoracic oncology standard (ARISTOT) and in accordance with data acquired from science as of March 19, 2018.
1. General
This guide presents the principles of diagnostic and therapeutic management of suspected or confirmed malignant pleural mesothelioma (MPM), as well as the professional aspects.
Malignant pleural mesothelioma (MPM) is a very aggressive cancerous tumor developed at the expense of the mesothelial cells of the pleura, pericardium or peritoneum.
It is a disease that is not very sensitive to chemotherapy but sensitive to radiotherapy.
Surgery is exceptionally possible. A rare disease, it affects 800 to 1000 people per year in France.
It is around 20 per million in Europe with a peak expected between 2015 and 2020.
There is a clear male predominance: 16 cases / 106 and 1.6 cases / 106 for women.
The average age is 60 years old.
Asbestos is the main causative agent of MPM. These are very small mineral fibers divided into 6 groups: chrysotile (serpentine), crocidolite, amosite, anthophyllite, atremolite and actinolite (the most toxic amphiboles).
Other risk factors are suspected: natural or industrial fibers (ceramic), ionizing radiation, simian virus 40 ...
Tobacco is not a risk factor but a multiplying factor.
Genetic factors are emerging, including the loss of expression of BAP1 or the protein associated with BRCA1.
MPM is very often an occupational disease in men (> 80%), much less in women (<40%) in whom domestic and environmental exposure predominates. The latency time is of the order of 30 to 50 years. No safety threshold has been demonstrated.
The assessment of exposure to asbestos must be carried out by questioning the patient, possibly supplemented by the use of professional or environmental questionnaires (SPLF identification questionnaire) and recorded in the medical file. Testing for asbestos fibers in alveolar lavage or lung tissue is not required for the medical management of MPM.
Natural history: lateralized chest pain revealing pleurisy, often recurrent. There is a progressive circumferential pleural thickening infiltrating and compressing the neighboring anatomical structures, invading the punctures, drainages and scars. The evolution appears essentially loco-regional. Metastases are however frequent in autopsy series. The median survival is 8 to 10 months after diagnosis.
Suspicion
clinic or
radiological
by MPM
Thoracoscopy
for diagnostic purposes
Option:
Symphysis
pleural
Thoraco-abdominal CT
after evacuation of
pleural effusion
eventual
Option:
Biopsies
pleural
needle
Declaration
mandatory
at the ARS
Histological confirmation
RCP
Option:
Radiotherapy*
early trips
thoracoscopy
or biospies to
the needle (ideally
within 15 days)
Treatment
FIVA **
Exposure
asbestos?
Disease
professional
* Radiotherapy within 2 to 6 weeks, 3x7 Gy,
tattoo biopsy paths
** Compensation Fund for Asbestos Victims
Yes
No
There is currently no medical benefit to performing systematic screening for MPM in patients exposed to asbestos, either radiologically or through biomarkers (soluble mesothelin related peptides, osteopontin, etc.). However, there are post-professional follow-up recommendations published in 2010 by the HAS.
Cf. Recommendations of the experts of the SPLF 2005 for the management of malignant pleural mesothelioma (2006); INCa documents (2011); HAS document (2010); Scherpereel (2010); Stahel (2010)
1.1. Notifiable disease
By decree n ° 2012-47 of January 16, 2012, malignant mesothelioma became the 31st notifiable disease in France.
Any new case of malignant mesothelioma, whatever its anatomical location, must be declared to the doctor of the Regional Health Agency (ARS) by the doctors who make the diagnosis.
The declaration forms are available on the website of the Institut de Veille Sanitaire (InVS): there are
a clinician component (Cerfa 14567 * 01)
a pathologist component (Cerfa 14568 * 01)
The patient must be registered in the National Mesothelioma Surveillance Program (PNSM), if it is in place locally and possibly registered in a register
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