Mésothéliome : les dispositifs en place en France « le réseau mésothéliome » 1998–2013

Abstract

Mesothelioma is a rare disease less than 0.3% of cancers in France, very aggressive and resistant to the majority of conventional therapies. Asbestos exposure is nearly the only recognized cause of mesothelioma in men observed in 80% of case. In 1990, the projections based on mortality predicted a raise of incidence in mesothelioma for the next three decades. Nowadays, the diagnosis of this cancer is based on pathology, but the histological presentation frequently heterogeneous, is responsible for numerous pitfalls and major problems of early detection toward effective therapy. Facing such a diagnostic, epidemiological and medico-legal context, a national and international multidisciplinary network has been progressively set up in order to answer to epidemiological survey, translational or academic research questions. Moreover, in response to the action of the French Cancer Program (action 23.1) a network of pathologists was organized for expert pathological second opinion using a standardized procedure of certification for mesothelioma diagnosis. We describe the network organization and show the results during this last 15years period of time from 1998-2013. These results show the major impact on patient's management, and confirm the interest of this second opinion to provide accuracy of epidemiological data, quality of medico-legal acknowledgement and accuracy of clinical diagnostic for the benefit of patients. We also show the impact of these collaborative efforts for creating a high quality clinicobiological, epidemiological and therapeutic data collection for improvement of the knowledge of this dramatic disease.

Management of malignant pleural mesothelioma: a French multicenter retrospective study (GFPC 0802 study)

“…The mean (median) numbers of cycles were 4.7 ± 1.7 [ 6 ], 3.5 ± 1.9 [ 3 ] and 3.1 ± 2 [ 3 ] in first, second and third lines respectively. The percentage of patients who responded to or were stabilized by treatment decreased over line, from 60.1 % in first line to 5.3 % in third line chemotherapy (Table 3 ).…”

“…The combination with pemetrexed resulted in longer overall survival (12.1 vs 9.3 months, p = 0.02), and increased response rates (41.3 vs 16.7 %, p < 0.0001). In our cohort, only 17.5 % of patients responded to this first-line chemotherapy, while the median number of cycles [ 6 ] was similar. According to the preliminary results of an Italian cohort of patients ( n = 322) receiving pemetrexed alone (28 %) or associated with cisplatin or carboplatin, the overall response rate was 28.5 % [ 24 ].…”

“…During the 2003–2007 5-year period, the pathological branch of the PNSM network (a mandatory, national review) analyzed 1348 pleural biopsies, among which 82 % were malignant mesotheliomas [ 6 ]. In this large sample of MPM, 80 % were epithelioïd, 6 % were sarcomatoid and 11 % were biphasic.…”

“…The number of MPM-related deaths is approximately 1100/year in men and 300/year in women in France [ 5 ] while the incidence ranges from less than 1/million for the general population to 50–100/million for at-risk sub-groups [ 5 ]. A national network has been created in 1998 (PNSM: Programme National de Surveillance des Mésothéliomes) for epidemiological observation, clinical research and organization of healthcare supply [ 6 ]. Apart from the well-known occupational exposure, environmental exposure to asbestos or other carcinogenic compounds remains to be explained while they seem to account for more and more cases [ 4 ].…”

Multicystic and diffuse malignant peritoneal mesothelioma in children

“…The diagnosis of DMPM was then confirmed by a reference center. We emphasize the need for centralization of immunohistological analysis of rare cancers to ensure expertise 28 …”