Clin Pediatr Hematol Oncol.  2024 Oct;31(2):29-35. 10.15264/cpho.2024.31.2.29.

Clinical Presentation, Adverse Prognostic Factors and Outcome Analysis of Pediatric Bone Tumors Treated at King Abdullah Specialized Children’s Hospital, Riyadh, Saudi Arabia - A Retrospective Review Over 20 Years

Affiliations
  • 1Department of Pediatric Hematology & Oncology, King Abdullah Specialized Children’s Hospital, Riyadh, Saudi Arabia
  • 2King Abdullah International Medical Research Centre, Riyadh, Saudi Arabia
  • 3King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia

Abstract

Background
Pediatric malignant bone tumors constitute an important subgroup of solid tumors and need comprehensive multidisciplinary care for optimal management. We aimed to review our local practice of managing malignant bone tumors and identify gaps to improve service.
Methods
We retrospectively reviewed disease characteristics and management of twenty-five pediatric patients with malignant bone tumors from Jan 2000 to Dec 2020.
Results
Median age at diagnosis was 8.75 years, male to female ratio of 0.8:1, and median follow-up was 3.6 years. Pain and swelling were the predominant symptoms at presentation, while lung was the most common metastatic site in Osteosarcoma (OS) and Ewing’s Sarcoma Family Tumors (ESFT). Metastatic disease was not associated with developing an event in OS (P=0.26) but was significantly associated with developing an event in the ESFT subgroup (P=0.002). There was no association of tumor necrosis of <90% or positive histological margins with developing an event in the entire cohort. The relapsed disease was associated with a mortality risk in the OS group (P=0.01). At the same time, it did not return significant results for this association in ESFT; however, a trend was noted for poor outcomes (P=0.09). Event-free survival and overall survival in OS were 54% and 69%, while in the ESFT group, they were 50% and 66%, respectively.
Conclusion
Metastatic disease was associated with developing relapsed disease in the ESFT cohort, while relapsed disease was associated with mortality in the OS cohort. Positive histological margins and <90% necrosis on resected tumor specimens did not result in poor survival. Multicenter collaboration at the national level is needed to improve the outcome of this disease group, which needs specialized multidisciplinary management.

Keyword

Pediatric bone tumors; Ewing sarcoma family of tumors; Osteosarcoma; Outcome analysis
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