Porret E, Madelaine J, Galateau-Sallé F, Bergot E, Zalcman G
Service de Pneumologie, Pôle Coeur-Poumons-Vaisseaux, Université Basse-Normandie, CHU de Caen, France.
Rev Mal Respir. 2007 Oct;24(8 Pt 2):6S157-64.
Malignant pleural mesothelioma (MPM) is a rare tumour due to occupational asbestos exposure. The incidence of MPM will continue to increase until 2020-2030. The incidence reaches 100 cases/million/year in occupationally exposed populations as opposed to 1 case/million/year in the general population, leading to 800 to 1,000 cases per year in France. The molecular carcinogenesis of MPM is incompletely understood but alterations to genes NF2, c-met, WT1 RASSF and p16 have been described. These genes are involved in cell invasion and motility, cell division and apoptosis control. Histological diagnosis remains difficult and depends on immunohistochemical analysis as described by the French Mesopath group. Clinical diagnosis relies on thoracoscopy and large pleural biopsies, with increasing use of CT-PET for the evaluation of disease extent. Therapeutic strategy includes prophylactic irradiation following drainage or thoracoscopy to prevent tumour nodule development along drainage channels and puncture sites. In selected patients, extensive extra-pleural pneumonectomy can be performed with curative intent. First line chemotherapy is based on a combination of pemetrexed and cisplatin that has demonstrated an improvement in overall survival and quality of life in phase 3 trials. Antiangiogenic agents such as bevacizumab (Avastatin) may be of interest but need to be tested in phase 3 trials. The Mesothelioma Avastatin Pemetrexed Study (MAPS) is ongoing, coordinated by the French Thoracic Cancer Intergroup (IFCT).
恶性胸膜间皮瘤(MPM)是一种因职业性接触石棉而引发的罕见肿瘤。MPM的发病率在2020年至2030年之前将持续上升。在职业暴露人群中,MPM的发病率达到每年100例/百万,而在普通人群中为每年1例/百万,这导致法国每年有800至1000例病例。MPM的分子致癌机制尚未完全明确,但已发现NF2、c-met、WT1、RASSF和p16等基因发生了改变。这些基因参与细胞侵袭与运动、细胞分裂以及细胞凋亡控制。组织学诊断依然困难,需依赖法国间皮瘤病理研究组所描述的免疫组织化学分析。临床诊断依靠胸腔镜检查和大型胸膜活检,CT-PET在评估疾病范围方面的应用日益增多。治疗策略包括在引流或胸腔镜检查后进行预防性放疗,以防止肿瘤结节沿引流通道和穿刺部位发展。对于部分患者,可进行广泛性胸膜外全肺切除术以达到治愈目的。一线化疗基于培美曲塞和顺铂联合方案,该方案在3期试验中已证明可改善总生存期和生活质量。抗血管生成药物如贝伐单抗(阿瓦斯汀)可能有效,但需要在3期试验中进行检验。间皮瘤阿瓦斯汀培美曲塞研究(MAPS)正在进行中,由法国胸癌研究组(IFCT)协调。