Abstract
Ewing sarcoma (ES) is a rare and aggressive disease that requires multidisciplinary treatment with the use of chemotherapy, radiotherapy, and surgery. Our retrospective study aimed to analyze the prognostic factors and treatment results in different age groups of patients. Between 1998 and 2018, 569 patients with ES were treated in two referral centers. The patients were divided into four age groups (≤10 years; 11–18 years; 19–25, and >25). The treatment results and prognostic factors were assessed for each group. For statistical analyses, we used the Chi2 test, the Kaplan–Meier estimator with a log-rank test, and the multivariate Cox model. Five-year overall survival (OS) rate was 56%. In the age subgroups: ≤10 years, 11–18 years, 19–25 years, and >25 years, the 5-year OS rates were 75%, 58%, 41%, and 52%, respectively. Favorable prognostic factors: female gender (p = 0.024), non-axial localization (p = 0.005), VIDE regimen (p < 0.001), and surgery as a local treatment (p < 0.001) dominated in the group ≤10 years. In multivariate analysis, male (HR = 1.53), axial localization (HR = 1.46), M1 status at presentation (HR = 2.64), and age > 10 years (HR = 2.29) were associated with shorter OS. The treatment results in ES are significantly better in children aged ≤10 years; the challenge is to provide therapy for adolescents and young adults. The diagnostics and treatment of ES patients must be provided in referral centers.
Highlights
Ewing sarcoma (ES) is the third most common primary malignant bone tumor that most often affects children and patients in the second decade of life [1,2]
We retrospectively analyzed the medical records of 569 patients with ES diagnosed and treated between 1998–2018 in two reference sarcoma centers involved in the Polish Sarcoma Group: 211 patients from MSCNRIO and 358 patients from the Mother and Child Institute, Warsaw
We reported a large cohort of pediatric and adult patients with ES treated in two reference sarcoma centers in Poland
Summary
Ewing sarcoma (ES) is the third most common primary malignant bone tumor that most often affects children and patients in the second decade of life [1,2]. Skeletal localization predominates especially in young populations (80%), while localization in the soft tissue is more common in older patients (>75%) [3,4]. The most common localizations of the tumor are the pelvic area and long bones of extremities with a predominance of the central femur [7,8]. About a quarter of patients have metastatic disease at the time of diagnosis [9]. Metastatic disease is most often found in the lungs (50%), bones (25%), and bone marrow (20%) [10,11]
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