Emri Salih A
Department of Chest Diseases, School of Medicine, Kemerburgaz University, Istanbul, Turkey.
Ann Transl Med. 2017 Jun;5(11):239. doi: 10.21037/atm.2017.04.06.
The epidemic of mesothelioma in Cappadocia, Turkey, is unprecedented in medical history. In three Cappadocian villages, Karain, Tuzkoy and "old" Sarihidir, about 50% of all deaths (including neonatal deaths and traffic fatalities) have been caused by mesothelioma. No other epidemic in medical history has caused such a high incidence of death. This is even more unusual when considering that (I) epidemics are caused by infectious agents, not cancer, and (II) mesothelioma is a rare cancer. World-wide mesothelioma incidence varies between 1/10 in areas with no asbestos industry to about 10-30/10 in areas with asbestos industry. This article reviews how the mesothelioma epidemic was discovered in Cappadocia by Dr. Baris (my mentor), how we initially linked the epidemic to erionite exposure, and later (with Dr. Carbone) to the interaction between genetic predisposition and environmental exposure. Our team's work had an important positive impact on the lives of those living in Cappadocia and also in many genetically predisposed families living around the world. I will discuss how the work that started in three remote Cappadocian villages led to the award of a NCI P01 grant to support our studies. Our studies proved that genetics modulates mineral fiber carcinogenesis and led to the discovery that carriers of germline mutations have a very high risk of developing mesothelioma and other malignancies. A new, very active field of research developed following our discoveries to elucidate the mechanism by which modulates mineral fiber carcinogenesis as well as to identify additional genes that when mutated increase the risk of mesothelioma and other environmentally related cancers. I am the only surviving member of this research team who saw all the phases of this research and I believe it is important to provide an accurate report, which hopefully will inspire others.
土耳其卡帕多西亚的间皮瘤疫情在医学史上是前所未有的。在卡帕多西亚的三个村庄，卡兰、图兹科伊和“老”萨里希迪尔，所有死亡（包括新生儿死亡和交通事故死亡）中约50%是由间皮瘤引起的。医学史上没有其他疫情导致过如此高的死亡率。考虑到以下两点，这就更加不同寻常了：（一）疫情是由传染因子引起的，而非癌症；（二）间皮瘤是一种罕见的癌症。全球间皮瘤发病率在没有石棉产业的地区为十万分之一，在有石棉产业的地区约为十万分之十至三十。本文回顾了巴里斯博士（我的导师）是如何在卡帕多西亚发现间皮瘤疫情的，我们最初是如何将疫情与毛沸石暴露联系起来的，以及后来（与卡尔博内博士一起）又是如何将其与遗传易感性和环境暴露之间的相互作用联系起来的。我们团队的工作对生活在卡帕多西亚的人们以及世界各地许多有遗传易感性的家庭的生活产生了重要的积极影响。我将讨论始于卡帕多西亚三个偏远村庄的这项工作是如何促成获得美国国立癌症研究所（NCI）P01资助以支持我们的研究的。我们的研究证明基因可调节矿物纤维致癌作用，并导致发现携带种系突变的个体患间皮瘤和其他恶性肿瘤的风险非常高。在我们的发现之后，一个全新且非常活跃的研究领域得以发展，以阐明基因调节矿物纤维致癌作用的机制，并识别出其他发生突变时会增加患间皮瘤和其他环境相关癌症风险的基因。我是这个研究团队中唯一见证了这项研究所有阶段的幸存者，我认为提供一份准确的报告很重要，希望它能激励他人。
