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Pediatric Axial Ewing Sarcoma: A Retrospective Population-Based Survival Analysis.

Brett A Hoffman1, Christopher Sanford, Alexander J Didier

  • 1University of Toledo College of Medicine and Life Sciences, Toledo, OH (Mr. Hoffman, Dr. Sanford, Mr. Didier, and Mr. Lassiter); Department of Orthopedic Surgery, Massachusetts General Hospital Boston, Boston, MA (Dr. Lozano-Calderon).

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This study highlights the critical role of multimodal therapy, including chemotherapy, for axial Ewing sarcomas in children. Early detection and timely treatment are vital for improving overall survival (OS) in these aggressive pediatric cancers.

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Area of Science:

  • Pediatric Oncology
  • Skeletal Tumors
  • Cancer Epidemiology

Background:

  • Axial Ewing sarcomas present unique challenges in pediatric patients due to aggressive behavior and potential neurovascular compromise.
  • Limited population-based data exist on treatment strategies and outcomes for axial Ewing sarcomas in children.

Purpose of the Study:

  • To conduct the first population-based analysis of treatment regimens for axial Ewing sarcomas in patients aged 1-24 years.
  • To evaluate the impact of different treatment modalities on cancer-specific survival and overall survival (OS).

Main Methods:

  • Utilized data from the Surveillance, Epidemiology, and End Results (SEER) database (2004-2019).
  • Categorized primary tumors into pelvic, thoracic, and vertebral sites.
  • Employed Chi-squared and Kaplan-Meier tests to analyze demographic, clinical, and treatment variables against patient survival.

Main Results:

  • Pelvic tumors were most frequent; vertebral tumors least frequent.
  • Treatment varied by site: chemotherapy/radiation for pelvic, chemotherapy/surgery/radiation for vertebral, and chemotherapy/surgery for thoracic tumors.
  • Overall survival (OS) was lowest for pelvic tumors (5-year OS: 70%) and highest for vertebral tumors (5-year OS: 77%).

Conclusions:

  • Chemotherapy-based multimodal therapy and early detection are crucial for treating pediatric axial Ewing sarcoma.
  • A significant decline in OS was noted between 5 and 10 years for thoracic tumors, with no improvement over previous cohorts.
  • A notable proportion of pelvic Ewing sarcoma patients did not receive radiation, indicating an unmet clinical need.