Journal
EUROPEAN JOURNAL OF PEDIATRICS
Volume 179, Issue 5, Pages 749-756Publisher
SPRINGER
DOI: 10.1007/s00431-019-03566-7
Keywords
Childhood cancer; Rare tumors; Pleuropulmonary blastoma; Cancer registry
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Funding
- European Union's Health Programme (2014-2020) [2013 12 07-CUP I94G 13,000,010,002, 777,336/PARTNER]
- Fondazione Celeghin
- Enfant, cancers et sante association
- Deutsche Kinderkrebsstiftung, Bonn, Germany [A2007/13DKS2009.08]
- Foerderkreis Krebskranke Kinder Stuttgart, Germany
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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.
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