Lettieri Sara, Bortolotto Chandra, Agustoni Francesco, Lococo Filippo, Lancia Andrea, Comoli Patrizia, Corsico Angelo G, Stella Giulia M
Department of Medical Sciences and Infective Diseases, Unit of Respiratory Diseases, IRCCS Policlinico San Matteo Foundation, University of Pavia Medical School, 27100 Pavia, Italy.
Department of Intensive Medicine, Unit of Radiology, IRCCS Policlinico San Matteo Foundation, University of Pavia Medical School, 27100 Pavia, Italy.
J Clin Med. 2021 Mar 3;10(5):1034. doi: 10.3390/jcm10051034.
Malignant pleural mesothelioma (MPM) is a rare and aggressive malignancy that most commonly affects the pleural lining of the lungs. It has a strong association with exposure to biopersistent fibers, mainly asbestos (80% of cases) and-in specific geographic regions-erionite, zeolites, ophiolites, and fluoro-edenite. Individuals with a chronic exposure to asbestos generally have a long latency with no or few symptoms. Then, when patients do become symptomatic, they present with advanced disease and a worse overall survival (about 13/15 months). The fibers from industrial production not only pose a substantial risk to workers, but also to their relatives and to the surrounding community. Modern targeted therapies that have shown benefit in other human tumors have thus far failed in MPM. Overall, MPM has been listed as orphan disease by the European Union. However, molecular high-throughput profiling is currently unveiling novel biomarkers and actionable targets. We here discuss the natural evolution, mainly focusing on the novel concept of molecular epidemiology. The application of innovative endpoints, quantification of genetic damages, and definition of genetic susceptibility are reviewed, with the ultimate goal to point out new tools for screening of exposed subject and for designing more efficient diagnostic and therapeutic strategies.
恶性胸膜间皮瘤（MPM）是一种罕见且侵袭性强的恶性肿瘤，最常累及肺的胸膜。它与接触生物持久性纤维密切相关，主要是石棉（80%的病例），在特定地理区域还与毛沸石、沸石、蛇绿岩和氟钙闪石有关。长期接触石棉的个体通常潜伏期很长，没有或只有很少的症状。然后，当患者出现症状时，往往已处于疾病晚期，总体生存率较差（约13/15个月）。工业生产中的纤维不仅对工人构成重大风险，对其亲属和周围社区也有风险。迄今为止，在其他人类肿瘤中显示出益处的现代靶向治疗在MPM中均告失败。总体而言，MPM已被欧盟列为罕见病。然而，分子高通量分析目前正在揭示新的生物标志物和可操作的靶点。我们在此讨论其自然演变，主要关注分子流行病学的新概念。对创新终点的应用、遗传损伤的量化以及遗传易感性的定义进行了综述，最终目的是指出用于筛查暴露个体以及设计更有效诊断和治疗策略的新工具。
