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.

Základní údaje

Originální název

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

Autoři

Vydání

European journal of pediatrics, New York, Springer, 2020, 0340-6199

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30209 Paediatrics

Stát vydavatele

Spojené státy

Utajení

není předmětem státního či obchodního tajemství

Odkazy

URL

Impakt faktor

Impact factor: 3.183

Označené pro přenos do RIV

Ano

Kód RIV

RIV/00216224:14110/20:00115983

Organizační jednotka

Lékařská fakulta

Klíčová slova anglicky

Childhood cancer; Rare tumors; Pleuropulmonary blastoma; Cancer registry

Štítky

14110321, rivok

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 31. 8. 2020 12:13, Mgr. Tereza Miškechová

Anotace

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 a 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, roč. 179, č. 5, s. 749-756. ISSN 0340-6199. Dostupné z: https://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 = {https://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 a 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, roč.~179, č.~5, s.~749-756. ISSN~0340-6199. Dostupné z: https://doi.org/10.1007/s00431-019-03566-7.

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