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Ewing sarcoma of the bone in children under 6 years of age
Maria Antonietta De Ioris1, Arcangelo Prete, Raffaele Cozza
1Haematology-Oncology Department, Ospedale Pediatrico Bambino Gesù-IRCCS, Rome, Italy.
Insights
Ewing Sarcoma Family Tumours (ESFT) are rare in young children. Children diagnosed with bone ESFT in Italy between 2000-2008 showed improved survival rates, particularly those with axial localization.
Area of Science:
- Pediatric Oncology
- Skeletal Tumors
- Cancer Epidemiology
Background:
- Ewing Sarcoma Family Tumours (ESFT) are rare in early childhood.
- This study focuses on ESFT of the bone in children under 6 years old in Italy.
Purpose of the Study:
- To report the clinical characteristics and outcomes of young children diagnosed with bone ESFT.
- To identify prognostic factors influencing survival in this pediatric population.
Main Methods:
- Retrospective review of 62 pediatric patients diagnosed with osseous ESFT from 1990-2008.
- Kaplan-Meier method for overall survival (OS) and progression-free survival (PFS) estimation.
- Multivariate analyses using Cox proportional hazards regression model.
Main Results:
- Axial primary localization occurred in 66% of patients, with chest wall involvement in 34%.
- Overall survival (OS) and progression-free survival (PFS) were significantly lower for patients with metastatic disease (38% and 21%, respectively) compared to localized disease (73% and 72%).
- Factors predicting worse survival included metastatic spread, axial/pelvic/spinal localization, treatment progression, and lack of surgery; patients treated from 2000-2008 had better survival (89% OS, 86% PFS).
Conclusions:
- Axial localization is the most common site for ESFT in pre-scholar children.
- Patients treated in the most recent period (2000-2008) demonstrated significantly improved outcomes.
- Early diagnosis and treatment advancements have led to better survival rates for pediatric bone ESFT.
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<0.01), while patients treated in the last decade had better survival (P = 0.002). In fact, the 5-year OS and PFS for patients diagnosed in the period 2000-2008 were 89% (95% CI 71-96%) and 86% (95% CI 66-94%), respectively.
Conclusion:
The axial localization is the most common site of ESFT in pre-scholar children. Patients treated in the most recent period have an excellent outcome.
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