Clinical Features, Prognostic Factors and Outcome of Children with Ewing Sarcoma: A Single-center Experience

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Al I., Demirağ B., Sabah D., Kamer S., Avşargil B. D., Argın M., ...More

GUNCEL PEDIATRI-JOURNAL OF CURRENT PEDIATRICS, vol.21, no.1, pp.60-68, 2023 (ESCI) identifier identifier

  • Publication Type: Article / Article
  • Volume: 21 Issue: 1
  • Publication Date: 2023
  • Doi Number: 10.4274/jcp.2023.46667
  • Journal Indexes: Emerging Sources Citation Index (ESCI), Scopus, Academic Search Premier, CAB Abstracts, CINAHL, EMBASE, Veterinary Science Database
  • Page Numbers: pp.60-68
  • Keywords: Ewing sarcoma, children, bone tumors, sarcoma
  • Istanbul Medipol University Affiliated: Yes


Introduction: Ewing sarcoma (ES) is a rare, aggressive, malignant tumor. It is the second most common malignant bone tumor in children. A total of 20-25% of patients are metastatic at the time of diagnosis. The survival rate for localized disease (LD) is approximately 70-74%. For metastatic disease (MD), it is about 30%. The most important prognostic factor affecting survival is the presence of MD at diagnosis. In this study, we investigated the clinical characteristics, treatment outcome, and factors affecting the prognosis and survival of patients followed up with the diagnosis of ES. Materials and Methods: Between 2007 and 2020, a total of 24 ES patients aged 0-18 years were retrospectively analyzed. Results: The most common complaint was pain and swelling in the lesion area (n=9), followed by pain (n=5), swelling (n=3), abdominal pain (n=2), shortness of breath (n=2), facial paralysis (n=1), spinal compression findings (leg pain and walking difficulty) (n=1) and hematuria (n=1). ES was bone-derived in 19 patients (79%). Of these, 14 had LD and 5 had MD at the time of diagnosis. Extraskeletal Ewing sarcomas (EES), was detected in five patients (21%) and derived from the kidney (n=1), rectus abdominis (n=1), left quadriceps femoris muscle (n=1), left upper thoracic region and lumbar region paraspinal muscles (n=2). The rate of MD was 25% (6/24) in the entire patient group. Disease progression was observed in three patients during treatment. Relapse at follow-up was observed in 6 of 19 patients in complete remission. The median time to relapse was 20 months (minimum 13, maximum 34 months) from diagnosis. The median survival of our patients after relapse was 14.5 months (minimum 6-maximum 27 months). Radiological response and histopathological response to induction therapy, presence of relapse or progression, and relapse site were found to be correlated with survival (Fisher’s Exact test p=0.02, 0.0047, <0.001, 0.001 respectiveley). Conclusion: ES is a cancer with high mortality and morbidity. Although the most common symptoms are pain and swelling, the symptoms may vary depending on the region from which the tumor originates. Response to induction therapy and the presence of relapse-progression are factors affecting prognosis. Treatment should be personalized to improve survival.