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

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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 languageEnglish
Article numbere53223
JournalPLoS One
Issue number1
Publication statusPublished - Jan 31 2013

ASJC Scopus subject areas

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


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