Pediatric Hematology and Oncology Division, Department of Women's and Children's Health, Padua University Hospital, Padua, Italy.
Klinikum Stuttgart-Olgahospital, Stuttgart Cancer Center, Zentrum für Kinder-, Jugend- und Frauenmedizin, Pediatrics 5 (Oncology, Hematology, Immunology), Stuttgart, Germany.
Eur J Pediatr. 2020 May;179(5):749-756. doi: 10.1007/s00431-019-03566-7. Epub 2020 Jan 4.
Very rare tumors (VRTs) account for up to 11% of childhood cancers. Dedicated national groups and registries only exist in some European countries. Pleuropulmonary blastoma (PPB) is a very rare intrathoracic pediatric tumor with a potentially severe prognosis. Due to its rarity, it sometimes goes unrecognized. We investigated PPB diagnostic capability and possible correlations between diagnostic performance and VRT-dedicated activities. The number of cases of PPB registered between 2000 and 2014 at pediatric oncology centers in Europe was compared with the number of expected cases. Data sources included VRT registries, population-based cancer registries, and hospital registries. Data were obtained for 25 countries, grouped into 4 geographical regions. The expected cases were 111, and the observed cases were 129. The observed-to-expected ratio was 1.86 for Northern Europe, 1.33 for Southern Europe, 1.22 for Central Europe, and 0.65 for Eastern Europe. More cases than expected were registered in all countries with an official VRT registry.Conclusion: The number of cases observed is consistent with expectations, but disparities exist across Europe. Difficulties in diagnosing PPB emerged in most Eastern countries. The incidence rate of PPB may be underestimated. The creation of VRT-dedicated groups and a European Registry for VRTs could help to reduce inequalities.What is Known:• Very rare pediatric tumors are often not recognized, despite representing almost 11% of childhood cancers .• Pleuropulmonary blastoma is a rare pediatric tumor with a poor prognosis.What is New:• The ability to diagnose and register pleuropulmonary blastoma varies in Europe.Registries dedicated to very rare pediatric tumors improve the diagnostic rates.• The incidence rate of pleuropulmonary blastoma may currently be underestimated.
非常罕见的肿瘤（VRTs）占儿童癌症的 11%。专门的国家团体和登记处仅存在于一些欧洲国家。胸膜肺胚细胞瘤（PPB）是一种非常罕见的胸腔内儿科肿瘤，具有潜在的严重预后。由于其罕见性，有时会被忽视。我们研究了 PPB 的诊断能力，以及诊断性能与 VRT 专用活动之间的可能相关性。将欧洲儿科肿瘤中心在 2000 年至 2014 年间登记的 PPB 病例数与预期病例数进行了比较。数据源包括 VRT 登记处、基于人群的癌症登记处和医院登记处。数据获取涵盖了 25 个国家，分为 4 个地理区域。预期病例为 111 例，观察病例为 129 例。观察到的与预期的比值为北欧 1.86，南欧 1.33，中欧 1.22，东欧 0.65。所有有官方 VRT 登记处的国家都登记了比预期更多的病例。结论：观察到的病例数量与预期相符，但欧洲各地存在差异。大多数东欧国家在诊断 PPB 方面存在困难。PPB 的发病率可能被低估。创建 VRT 专用组和 VRT 欧洲登记处可能有助于减少不平等。已知：• 非常罕见的儿科肿瘤经常未被识别，尽管它们占儿童癌症的近 11%。• 胸膜肺胚细胞瘤是一种预后不良的罕见儿科肿瘤。新内容：• 欧洲在诊断和登记胸膜肺胚细胞瘤方面的能力存在差异。专门针对非常罕见的儿科肿瘤的登记处提高了诊断率。• 胸膜肺胚细胞瘤的发病率目前可能被低估。
