Galateau-Sallé Françoise, Gilg Soit Ilg A, Le Stang N, Brochard P, Pairon J C, Astoul P, Frenay C, Blaizot G, Chamming's S, Ducamp S, Rousvoal T, de Quillacq A, Abonnet V, Abdalsamad I, Begueret H, Brambilla E, Capron F, Copin M C, Danel C, de Lajartre A Y, Foulet-Roge A, Garbe L, Groussard O, Giusiano S, Hofman V, Lantuejoul S, Piquenot J M, Rouquette I, Sagan C, Thivolet-Bejui F, Vignaud J M, Scherpereel A, Jaurand M C, Jean D, Hainaut P, Chérié-Challine L, Goldberg M, Luce D, Imbernon E
Centre national référent cancers rares MESOPATH « mésothéliomes malins pleuraux et tumeurs péritonéales rares », CHU Caen Côte-de-Nacre, 14033 Caen cedex 9, France; Registre MESONAT, CHU Caen Côte-de-Nacre, 14033 Caen cedex 9, France.
Département santé travail, institut de veille sanitaire, 12, rue du Val-d'Osne, 94415 Saint-Maurice cedex, France.
Ann Pathol. 2014 Feb;34(1):51-63. doi: 10.1016/j.annpat.2014.01.009. Epub 2014 Feb 26.
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.
间皮瘤是一种罕见疾病，在法国占癌症病例不到0.3%，极具侵袭性且对大多数传统疗法耐药。石棉暴露几乎是男性间皮瘤唯一公认的病因，80%的病例都观察到这一点。1990年，基于死亡率的预测表明，未来三十年中间皮瘤发病率会上升。如今，这种癌症的诊断基于病理学，但组织学表现常常具有异质性，这导致了许多陷阱以及早期检测以实现有效治疗方面的重大问题。面对这样的诊断、流行病学和法医学背景，一个国家和国际多学科网络已逐步建立起来，以应对流行病学调查、转化或学术研究问题。此外，为响应法国癌症计划（行动23.1）的行动，组织了一个病理学家网络，通过标准化的间皮瘤诊断认证程序提供专家病理二次诊断意见。我们描述了该网络的组织情况，并展示了1998年至2013年这过去15年期间的结果。这些结果显示了对患者管理的重大影响，并证实了这种二次诊断意见在提供准确的流行病学数据、法医学认定质量和临床诊断准确性以造福患者方面的益处。我们还展示了这些合作努力对创建高质量临床生物学、流行病学和治疗数据收集的影响，以增进对这种严重疾病的了解。
