Ewing Sarcoma of the Bone in Children under 6 Years of Age

Maria Antonietta De Ioris; Arcangelo Prete; Raffaele Cozza; Marta Podda; Carla Manzitti; Andrea Pession; Elisabetta Schiavello; Benedetta Contoli; Rita Balter; Franca Fagioli; Gianni Bisogno; Loredana Amoroso; Franco Locatelli; Roberto Luksch

doi:10.1371/journal.pone.0053223

Ewing Sarcoma of the Bone in Children under 6 Years of Age

Maria Antonietta De Ioris, Arcangelo Prete, Raffaele Cozza, Marta Podda, Carla Manzitti, Andrea Pession, Elisabetta Schiavello, Benedetta Contoli, Rita Balter, Franca Fagioli, Gianni Bisogno, Loredana Amoroso, Franco Locatelli, Roberto Luksch

Research output: Contribution to journal › Article › peer-review

Abstract

Background: Ewing Sarcoma Family Tumours (ESFT) are rare in early childhood. The aim of this study was to report the clinical characteristics and outcome of children under 6 years of age affected by ESFT of the bone in Italy. Methods: The records of all the children diagnosed with osseous ESFT in centres members of the Associazione Italiana di Ematologia ed Oncologia Pediatrica (AIEOP) from 1990 to 2008 were reviewed. The Kaplan-Meier method was used for estimating overall and progression-free survival (OS, PFS) curves; multivariate analyses were performed using Cox proportional hazards regression model. Results: This study includes 62 patients. An axial primary localization was present in 66% of patients, with the primary site in the chest wall in 34%. Fourteen (23%) patients presented metastatic disease. The 5-year OS and PFS were 73% (95% confidence interval, CI, 58-83%) and 72% (95% CI 57-83%) for patients with localized disease and 38% (95% CI 17-60%) and 21% (95% CI 5-45%) for patients with metastatic disease. Metastatic spread, skull/pelvis/spine primary localization, progression during treatment and no surgery predicted worse survival (P

Original language	English
Article number	e53223
Journal	PLoS One
Volume	8
Issue number	1
DOIs	https://doi.org/10.1371/journal.pone.0053223
Publication status	Published - Jan 31 2013

ASJC Scopus subject areas

Agricultural and Biological Sciences(all)
Biochemistry, Genetics and Molecular Biology(all)
Medicine(all)

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10.1371/journal.pone.0053223

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title = "Ewing Sarcoma of the Bone in Children under 6 Years of Age",

abstract = "Background: Ewing Sarcoma Family Tumours (ESFT) are rare in early childhood. The aim of this study was to report the clinical characteristics and outcome of children under 6 years of age affected by ESFT of the bone in Italy. Methods: The records of all the children diagnosed with osseous ESFT in centres members of the Associazione Italiana di Ematologia ed Oncologia Pediatrica (AIEOP) from 1990 to 2008 were reviewed. The Kaplan-Meier method was used for estimating overall and progression-free survival (OS, PFS) curves; multivariate analyses were performed using Cox proportional hazards regression model. Results: This study includes 62 patients. An axial primary localization was present in 66% of patients, with the primary site in the chest wall in 34%. Fourteen (23%) patients presented metastatic disease. The 5-year OS and PFS were 73% (95% confidence interval, CI, 58-83%) and 72% (95% CI 57-83%) for patients with localized disease and 38% (95% CI 17-60%) and 21% (95% CI 5-45%) for patients with metastatic disease. Metastatic spread, skull/pelvis/spine primary localization, progression during treatment and no surgery predicted worse survival (P",

author = "{De Ioris}, {Maria Antonietta} and Arcangelo Prete and Raffaele Cozza and Marta Podda and Carla Manzitti and Andrea Pession and Elisabetta Schiavello and Benedetta Contoli and Rita Balter and Franca Fagioli and Gianni Bisogno and Loredana Amoroso and Franco Locatelli and Roberto Luksch",

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T1 - Ewing Sarcoma of the Bone in Children under 6 Years of Age

AU - De Ioris, Maria Antonietta

AU - Prete, Arcangelo

AU - Cozza, Raffaele

AU - Podda, Marta

AU - Manzitti, Carla

AU - Pession, Andrea

AU - Schiavello, Elisabetta

AU - Contoli, Benedetta

AU - Balter, Rita

AU - Fagioli, Franca

AU - Bisogno, Gianni

AU - Amoroso, Loredana

AU - Locatelli, Franco

AU - Luksch, Roberto

PY - 2013/1/31

Y1 - 2013/1/31

N2 - Background: Ewing Sarcoma Family Tumours (ESFT) are rare in early childhood. The aim of this study was to report the clinical characteristics and outcome of children under 6 years of age affected by ESFT of the bone in Italy. Methods: The records of all the children diagnosed with osseous ESFT in centres members of the Associazione Italiana di Ematologia ed Oncologia Pediatrica (AIEOP) from 1990 to 2008 were reviewed. The Kaplan-Meier method was used for estimating overall and progression-free survival (OS, PFS) curves; multivariate analyses were performed using Cox proportional hazards regression model. Results: This study includes 62 patients. An axial primary localization was present in 66% of patients, with the primary site in the chest wall in 34%. Fourteen (23%) patients presented metastatic disease. The 5-year OS and PFS were 73% (95% confidence interval, CI, 58-83%) and 72% (95% CI 57-83%) for patients with localized disease and 38% (95% CI 17-60%) and 21% (95% CI 5-45%) for patients with metastatic disease. Metastatic spread, skull/pelvis/spine primary localization, progression during treatment and no surgery predicted worse survival (P

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Ewing Sarcoma of the Bone in Children under 6 Years of Age

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