Trama Annalisa, Bernasconi Alice, Cañete Adela, Carulla Marià, Daubisse-Marliac Laetitia, Rossi Silvia, De Angelis Roberta, Sanvisens Arantza, Katalinic Alexander, Paapsi Keiu, Went Philip, Mousavi Mohsen, Blum Marcel, Eberle Andrea, Lamy Sébastien, Capocaccia Riccardo, Didonè Fabio, Botta Laura
Evaluative Epidemiology Unit, Department of Epidemiology and Data Science, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Via Venenzian, 1, 20133 Milan, Italy.
Evaluative Epidemiology Unit, Department of Epidemiology and Data Science, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Via Venenzian, 1, 20133 Milan, Italy.
Eur J Cancer. 2025 Jan;214:115147. doi: 10.1016/j.ejca.2024.115147. Epub 2024 Nov 27.
Rare cancers correspond to approximately 200 clinical entities, which can be grouped into 12 families. Updated data are available for childhood and haematological cancers, ie, for only two of the 12 families of rare cancer. We provide incidence and survival for the remaining ten families of rare adult solid cancers (RAC), across 29 EU Member States and over time. We also evaluate the association between resources invested in health and survival from RACs.
We used the EUROCARE-6 database, which includes data from 108 cancer registries from 29 countries. We calculated incidence rates (IR) and 5-year relative survival (RS) for cases diagnosed during 2006-2013. We calculated 5-year RS in the follow-up period 2010-2014 using the period approach (last follow-up: December 31, 2014). We estimated changes in 5-year RS and IR over the period 2000-2013. We used a forest plot to report the differences in RS among countries with the highest and lowest health spending.
RACs are heterogeneous in terms of incidence, survival, sex, and age distribution. Several RACs (eg, those of the hypopharynx, small intestine, and trachea) still have a 5-year RS < 30 %, which is not improving. Survival differs among European countries and is higher in countries with the greatest investments in health. The incidence of smoking-related RACs is decreasing but rising in HPV-related RACs.
Investments in health and healthcare networks at national and European level can help increase the survival of RACs, especially those requiring centralisation of care (eg, bone sarcomas, penile cancer). These investments are critical considering that survival from RACs is not significantly improving. Our results unmask the heterogeneity of RACs, which needs to be considered in clinical trial design. Finally, our findings support the importance of prevention strategies for known risk factors such as smoking.
罕见癌症约有200种临床类型，可分为12个类别。目前已有关于儿童癌症和血液系统癌症的最新数据，即12类罕见癌症中仅有两类的相关数据。我们提供了29个欧盟成员国中其余十类成人罕见实体癌（RAC）的发病率和生存率，并分析了随时间的变化情况。我们还评估了卫生投入资源与RAC生存率之间的关联。
我们使用了EUROCARE - 6数据库，该数据库包含来自29个国家108个癌症登记处的数据。我们计算了2006 - 2013年期间确诊病例的发病率（IR）和5年相对生存率（RS）。我们采用时期法（最后随访时间：2014年12月31日）计算了2010 - 2014年随访期间的5年RS。我们估计了2000 - 2013年期间5年RS和IR的变化。我们使用森林图来报告卫生支出最高和最低国家之间的RS差异。
RAC在发病率、生存率、性别和年龄分布方面存在异质性。几种RAC（如下咽癌、小肠癌和气管癌）的5年RS仍低于30%，且没有改善。欧洲国家之间的生存率存在差异，在卫生投入最大的国家生存率更高。与吸烟相关的RAC发病率在下降，但与人乳头瘤病毒（HPV）相关的RAC发病率在上升。
国家和欧洲层面在卫生及医疗保健网络方面的投入有助于提高RAC的生存率，尤其是那些需要集中治疗的癌症（如骨肉瘤、阴茎癌）。鉴于RAC的生存率没有显著提高，这些投入至关重要。我们的研究结果揭示了RAC的异质性，这在临床试验设计中需要予以考虑。最后，我们的研究结果支持了针对吸烟等已知风险因素采取预防策略的重要性。
