BACKGROUND: Ewing sarcoma (EwS) is a rare and highly malignant bone tumor primarily affecting children, adolescents, and young adults. The pelvis, trunk, and lower extremities are the most common sites, while EwS of the sacrum as a primary site is very rare, and only few studies focusing on this location are published. Due to the anatomical condition, local treatment is challenging in sacral malignancies. We analyzed factors that might influence the outcome of patients suffering from sacral EwS. METHODS: We retrospectively analyzed data of the GPOH EURO-E.W.I.N.G 99 trial and the EWING 2008 trial, with a cohort of 124 patients with localized or metastatic sacral EwS. The study endpoints were overall survival (OS) and event-free survival (EFS). OS and EFS were calculated using the Kaplan-Meier method, and univariate comparisons were estimated using the log-rank test. Hazard ratios (HRs) with respective 95% confidence intervals (CIs) were estimated in a multivariable Cox regression model. RESULTS: The presence of metastases (3y-EFS: 0.33 vs. 0.68; P < 0.001; HR = 3.4, 95% CI 1.7 to 6.6; 3y-OS: 0.48 vs. 0.85; P < 0.001; HR = 4.23, 95% CI 1.8 to 9.7), large tumor volume (≥200 ml) (3y-EFS: 0.36 vs. 0.69; P=0.02; HR = 2.1, 95% CI 1.1 to 4.0; 3y-OS: 0.42 vs. 0.73; P=0.04; HR = 2.1, 95% CI 1.03 to 4.5), and age ≥18 years (3y-EFS: 0.41 vs. 0.60; P=0.02; HR = 2.6, 95% CI 1.3 to 5.2; 3y-OS: 0.294 vs. 0.59; P=0.01; HR = 2.92, 95% CI 1.29 to 6.6) were revealed as adverse prognostic factors. CONCLUSION: Young age seems to positively influence patients` survival, especially in patients with primary metastatic disease. In this context, our results support other studies, stating that older age has a negative impact on survival. Tumor volume, metastases, and the type of local therapy modality have an impact on the outcome of sacral EwS. Level of evidence: Level 2. This trial is registered with NCT00020566 and NCT00987636.

Center for Pediatric Oncology and Hematology Vilnius University Hospital Santaros Klinikos Vilnius University Vilnius Lithuania

Charles University Motol Child Ren's Hospital Prague Czech Republic

Childhood Cancer Center Queen Silvia Children's Hospital Gothenburg Sweden

Chris O'Brien Lifehouse Camperdown Australia

Clinic for Particle Therapy West German Proton Beam Centre University Hospital Essen West German Cancer Centre German Cancer Research Centre Essen Germany

Clinic of Tumororthopedics University Hospital Essen West German Cancer Centre Essen Germany

Clinics of Children's Diseases Faculty of Medicine Vilnius University Vilnius Lithuania

Cliniques Universitaires Saint Luc Department of Pediatric Hematology and Oncology Université Catholique de Louvain Brussels Belgium

Department of Oncology Hematology University Children's Hospital Basel Basel Switzerland

Department of Pediatric Hematology and Oncology University Children's Hospital Münster West German Cancer Center Network Münster Germany

Faculty of Medicine and Health University of Sydney Camperdown Australia

Gerhard Domagk Institute for Pathology University Hospital Muenster West German Cancer Center Network Muenster Germany

German Cancer Consortium Partnersite Essen Germany

HUS Helsinki University Hospital New Children's Hospital Div Hematology and Stem Cell Transplantation Helsinki Finland

Leiden University Medical Center Department of Medical Oncology Leiden NL USA

Lung Clinic Department of Thoracic Surgery Cologne Merheim Hospital University of Witten Herdecke Witten Germany

Mother and Child Institute Department of Oncology and Surgical Oncology for Children and Youth Warsaw Poland

Patient Representative St Josef's Hospital Bochum University Hospital Bochum Germany

Pediatrics 3 West German Cancer Center University Hospital Essen University of Duisburg Essen Essen Germany

Princess Máxima Center for Pediatric Oncology Department of Solid Tumors Utrecht Netherlands

Radiotherapy and Radiooncology University Hospital Muenster West German Cancer Center Network Muenster Germany

Velkey László Child's Health Center Borsod Abaúj Zemplén County University Teaching Hospital Miskolc Hungary

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ClinicalTrials.gov
NCT00987636, NCT00020566