Inequalities in diagnosis and registration of pediatric very
rare tumors: a European study on pleuropulmonary blastoma

J 2020

Inequalities in diagnosis and registration of pediatric very rare tumors: a European study on pleuropulmonary blastoma

GRIGOLETTO, V., A. TAGARELLI, M. SPARBER-SAUER, E. KOSCIELNIAK, D. ORBACH et. al.

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

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30209 Paediatrics

Country of publisher

United States of America

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

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

Abstract

V originále

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.

Citovat

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.

@article{1670351,
   author = {Grigoletto, V. and Tagarelli, A. and SparberandSauer, M. and Koscielniak, E. and Orbach, D. and Duplan, M. and StachowiczandStencel, T. and Bien, E. and LopezandAlmaraz, R. and BenandAmi, T. and Pourtsidis, A. and Osterlundh, G. and Mazánek, Pavel and Zsiros, J. and Farinha, N. and Ferrari, A. and Bisogno, G.},
   article_location = {New York},
   article_number = {5},
   doi = {http://dx.doi.org/10.1007/s00431-019-03566-7},
   keywords = {Childhood cancer; Rare tumors; Pleuropulmonary blastoma; Cancer registry},
   language = {eng},
   issn = {0340-6199},
   journal = {European journal of pediatrics},
   title = {Inequalities in diagnosis and registration of pediatric very rare tumors: a European study on pleuropulmonary blastoma},
   url = {https://link.springer.com/article/10.1007%2Fs00431-019-03566-7},
   volume = {179},
   year = {2020}
}

TY  - JOUR
ID  - 1670351
AU  - Grigoletto, V. - Tagarelli, A. - Sparber-Sauer, M. - Koscielniak, E. - Orbach, D. - Duplan, M. - Stachowicz-Stencel, T. - Bien, E. - Lopez-Almaraz, R. - Ben-Ami, T. - Pourtsidis, A. - Osterlundh, G. - Mazánek, Pavel - Zsiros, J. - Farinha, N. - Ferrari, A. - Bisogno, G.
PY  - 2020
TI  - Inequalities in diagnosis and registration of pediatric very rare tumors: a European study on pleuropulmonary blastoma
JF  - European journal of pediatrics
VL  - 179
IS  - 5
SP  - 749-756
EP  - 749-756
PB  - Springer
SN  - 03406199
KW  - Childhood cancer
KW  - Rare tumors
KW  - Pleuropulmonary blastoma
KW  - Cancer registry
UR  - https://link.springer.com/article/10.1007%2Fs00431-019-03566-7
L2  - https://link.springer.com/article/10.1007%2Fs00431-019-03566-7
N2  - 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.
ER  -

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. \textit{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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