Department of Preventive and Predictive Medicine, Fondazione IRCSS, Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy.
Eur J Cancer. 2012 Jul;48(10):1425-33. doi: 10.1016/j.ejca.2011.12.027. Epub 2012 Feb 20.
Embryonal cancers are a heterogeneous group of rare cancers which mainly occur in children and adolescents. The aim of the present study was to estimate the burden (incidence, prevalence, survival and proportion of cured) for the principal embryonal cancers in Europe (EU27), using population-based data from cancer registries (CRs) participating in RARECARE. We identified 3322 cases diagnosed from 1995 to 2002 (latest period for which data are available): 44% neuroblastoma, 35% nephroblastoma, 13% retinoblastoma and 6% hepatoblastoma. Very few cases of pulmonary blastoma (43 cases) and pancreatoblastoma (seven cases) were diagnosed. About 2000 new embryonal cancers were estimated every year in EU27, for an annual incidence rate of 4 per million (1.8 neuroblastoma, 1.4 nephroblastoma, and 0.5 retinoblastoma); 91% of cases occurred in patients under 15 years. Five-year relative survival for all embryonal cancers was 80% (99% retinoblastoma, 90% nephroblastoma, 71% hepatoblastoma and 68% neuroblastoma). Overall survival was lower in adolescents and adults than in those under 15 years. The cure rate was estimated at 80%. Slightly less than 40,000 persons were estimated alive in EU27 with a diagnosis of embryonal cancer in 2008. Nephroblastoma was the most prevalent (18,150 cases in EU27), followed by neuroblastoma (12,100), retinoblastoma (5200), hepatoblastoma (2700) and pulmonary blastoma (614). This is the first study to delineate the embryonal cancer burden in Europe by age, sex and European region. Survival/cure rate is generally high, but there are considerable gaps in our understanding of the natural histories of these rare diseases particularly in adults.
胚胎性癌是一组罕见的癌症，主要发生在儿童和青少年中。本研究的目的是使用参与 RARECARE 的癌症登记处（CR）的基于人群的数据，估算欧洲（EU27）主要胚胎性癌的负担（发病率、患病率、生存率和治愈率）。我们确定了 1995 年至 2002 年期间诊断的 3322 例病例（可获得最新数据的时期）：44%为神经母细胞瘤，35%为肾母细胞瘤，13%为视网膜母细胞瘤，6%为肝母细胞瘤。诊断出的肺胚细胞瘤（43 例）和胰腺胚细胞瘤（7 例）非常少见。EU27 每年估计有 2000 例新的胚胎性癌，发病率为每百万人口 4 例（1.8 例神经母细胞瘤，1.4 例肾母细胞瘤和 0.5 例视网膜母细胞瘤）；91%的病例发生在 15 岁以下的患者中。所有胚胎性癌的 5 年相对生存率为 80%（99%的视网膜母细胞瘤，90%的肾母细胞瘤，71%的肝母细胞瘤和 68%的神经母细胞瘤）。青少年和成年人的总生存率低于 15 岁以下的人群。治愈率估计为 80%。2008 年，EU27 估计有不到 4 万人被诊断患有胚胎性癌，目前仍存活。肾母细胞瘤（EU27 有 18150 例）最为常见，其次是神经母细胞瘤（12100 例）、视网膜母细胞瘤（5200 例）、肝母细胞瘤（2700 例）和肺胚细胞瘤（614 例）。这是首次按年龄、性别和欧洲区域划分欧洲胚胎性癌负担的研究。生存率/治愈率通常较高，但我们对这些罕见疾病的自然史的了解仍存在相当大的差距，特别是在成年人中。
