GRIGOLETTO, V., A. TAGARELLI, M. SPARBER-SAUER, E. KOSCIELNIAK, D. ORBACH, M. DUPLAN, T. STACHOWICZ-STENCEL, E. BIEN, R. LOPEZ-ALMARAZ, T. BEN-AMI, A. POURTSIDIS, G. OSTERLUNDH, Pavel MAZÁNEK, J. ZSIROS, N. FARINHA, A. FERRARI and G. BISOGNO. Inequalities in diagnosis and registration of pediatric very rare tumors: a European study on pleuropulmonary blastoma. European journal of pediatrics. New York: Springer, 2020, vol. 179, No 5, p. 749-756. ISSN 0340-6199. Available from: https://dx.doi.org/10.1007/s00431-019-03566-7.
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Basic information
Original name Inequalities in diagnosis and registration of pediatric very rare tumors: a European study on pleuropulmonary blastoma
Authors GRIGOLETTO, V. (380 Italy, guarantor), A. TAGARELLI (380 Italy), M. SPARBER-SAUER (276 Germany), E. KOSCIELNIAK (276 Germany), D. ORBACH (250 France), M. DUPLAN (250 France), T. STACHOWICZ-STENCEL (616 Poland), E. BIEN (616 Poland), R. LOPEZ-ALMARAZ (724 Spain), T. BEN-AMI (376 Israel), A. POURTSIDIS (300 Greece), G. OSTERLUNDH (752 Sweden), Pavel MAZÁNEK (203 Czech Republic, belonging to the institution), J. ZSIROS (528 Netherlands), N. FARINHA (620 Portugal), A. FERRARI (380 Italy) and G. BISOGNO (380 Italy).
Edition European journal of pediatrics, New York, Springer, 2020, 0340-6199.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30209 Paediatrics
Country of publisher United States of America
Confidentiality degree is not subject to a state or trade secret
WWW URL
Impact factor Impact factor: 3.183
RIV identification code RIV/00216224:14110/20:00115983
Organization unit Faculty of Medicine
Doi http://dx.doi.org/10.1007/s00431-019-03566-7
UT WoS 000505418200003
Keywords in English Childhood cancer; Rare tumors; Pleuropulmonary blastoma; Cancer registry
Tags 14110321, rivok
Tags International impact, Reviewed
Changed by Changed by: Mgr. Tereza Miškechová, učo 341652. Changed: 31/8/2020 12:13.
Abstract
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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