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
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Very rare tumor
Congenital pulmonary airway malformation
Pediatric Rare Tumors Network–European Registry
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The authors wish to thank Brigitte Lacour, Alexandra Kolenova, Maja Cesen Mazic, Christine Devalck, Catherine Rechnitzer, Heidi Glosli, Dobrin Konstantinov, Jelena Rascon, Jelena Roganovic, and Zsuzsanna Jakab for their contribution to data collection in their respective countries. We also extend our sincere thanks to Beatrice Coppadoro and Ilaria Zanetti for their help with data analysis, Serena Mancini for managing the PARTNER project, and Silvia Sorbara for managing the ExPO-r-Net project.
This work stems from the activities of the ExPO-r-Net project (Project 2013 12 07-CUP I94G 13,000,010,002) and was undertaken as part of the “777,336/PARTNER” project, which received funding from the European Union’s Health Programme (2014–2020). The content of this paper represents the views of the author only and is his/her sole responsibility; it can not be considered to reflect the views of the European Commission and/or the Consumers, Health, Agriculture and Food Executive Agency or any other body of the European Union. The European Commission and the Agency do not accept any responsibility for use that may be made of the information it contains. The Italian TREP Registry is supported by the Fondazione Celeghin. The French Fracture database is supported by the “Enfant, cancers et santé” association. The German (SoTiSaR), registry was supported by the Deutsche Kinderkrebsstiftung, Bonn, Germany, grant no. A2007/13DKS2009.08, and by the Foerderkreis Krebskranke Kinder Stuttgart, Germany.
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Grigoletto, V., Tagarelli, A., Sparber-Sauer, M. et al. Inequalities in diagnosis and registration of pediatric very rare tumors: a European study on pleuropulmonary blastoma. Eur J Pediatr (2020) doi:10.1007/s00431-019-03566-7
- Childhood cancer
- Rare tumors
- Pleuropulmonary blastoma
- Cancer registry