Department of Registry and Research, Comprehensive Cancer Centre The Netherlands, PO Box 330, 9700 AH Groningen, The Netherlands.
Eur J Cancer. 2012 May;48(7):949-60. doi: 10.1016/j.ejca.2012.02.047. Epub 2012 Mar 9.
Rare thoracic cancers include those of the trachea, thymus and mesothelioma (including peritoneum mesothelioma). The aim of this study was to describe the incidence, prevalence and survival of rare thoracic tumours using a large database, which includes cancer patients diagnosed from 1978 to 2002, registered in 89 population-based cancer registries (CRs) and followed-up to 31st December 2003. Over 17,688 cases of rare thoracic cancers were selected based on the list of the RACECARE project. Mesothelioma was the most common tumour (19 per million per year) followed by epithelial tumours of the trachea and thymus (1.3 and 1.7, respectively). The age standardised incidence rates of epithelial tumours of the trachea was double in Eastern and Southern Europe versus the other European regions: 2 per million per year. Epithelial tumours of the thymus had the lowest incidence in Northern and Eastern Europe and UK and Ireland(1) and somewhat higher incidence in Central and Southern Europe.(2) Highest incidence in mesothelioma was seen in UK and Ireland(23) and lowest in Eastern Europe.(4) Patients with tumours of the thymus had the best prognosis (1-year survival 85%, 66% at 5 years). Five year survival was lowest for the mesothelioma 5% compared to 14% of patients with tumours of the trachea. Mesothelioma was the most prevalent rare cancer (12,000 cases), followed by thymus (7000) and trachea (1400). Cancer Registry (CR) data play an important role in revealing the burden of rare thoracic cancers and monitoring the effect of regulations on asbestos use and smoking related policies.
罕见的胸部肿瘤包括气管、胸腺和间皮瘤（包括腹膜间皮瘤）。本研究的目的是使用一个大型数据库描述罕见胸部肿瘤的发病率、患病率和生存率，该数据库包括 1978 年至 2002 年诊断的癌症患者，登记在 89 个基于人群的癌症登记处（CR）中，并随访至 2003 年 12 月 31 日。根据 RACECARE 项目的清单，选择了超过 17688 例罕见的胸部癌症病例。间皮瘤是最常见的肿瘤（每年每百万 19 例），其次是气管和胸腺的上皮肿瘤（分别为 1.3 和 1.7）。气管上皮肿瘤的年龄标准化发病率在东欧和南欧是其他欧洲地区的两倍：每年每百万 2 例。胸腺上皮肿瘤在北欧和东欧以及英国和爱尔兰的发病率最低（1），在中欧和南欧的发病率略高（2）。间皮瘤的发病率最高见于英国和爱尔兰（23），最低见于东欧（4）。胸腺肿瘤患者的预后最好（1 年生存率 85%，5 年生存率 66%）。间皮瘤患者的 5 年生存率最低，为 5%，而气管肿瘤患者的 5 年生存率为 14%。间皮瘤是最常见的罕见癌症（12000 例），其次是胸腺（7000 例）和气管（1400 例）。癌症登记处（CR）数据在揭示罕见胸部癌症的负担和监测石棉使用法规和与吸烟相关政策的效果方面发挥着重要作用。
