Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: A report from the Children's Oncology Group

Thomas Cash, Elizabeth McIlvaine, Mark D. Krailo, Stephen L. Lessnick, Elizabeth R. Lawlor, Nadia Laack, Joel Sorger, Neyssa Marina, Holcombe E. Grier, Linda Granowetter, Richard B. Womer, Steven G. DuBois

Research output: Contribution to journalArticle

28 Scopus citations

Abstract

Background: The prognostic significance of having extraskeletal (EES) versus skeletal Ewing sarcoma (ES) in the setting of modern chemotherapy protocols is unknown. The purpose of this study was to compare the clinical characteristics, biologic features, and outcomes for patients with EES and skeletal ES. Methods: Patients had localized ES and were treated on two consecutive protocols using five-drug chemotherapy (INT-0154 and AEWS0031). Patients were analyzed based on having an extraskeletal (n = 213) or skeletal (n = 826) site of tumor origin. Event-free survival (EFS) was estimated using the Kaplan–Meier method, compared using the log-rank test, and modeled using Cox multivariate regression. Results: Patients with extraskeletal ES (EES) were more likely to have axial tumors (72% vs. 55%; P < 0.001), less likely to have tumors >8 cm (9% vs. 17%; P < 0.01), and less likely to be white (81% vs. 87%; P < 0.001) compared to patients with skeletal ES. There was no difference in key genomic features (type of EWSR1 translocation, TP53 mutation, CDKN2A mutation/loss) between groups. After controlling for age, race, and primary site, EES was associated with superior EFS (hazard ratio = 0.69; 95% confidence interval: 0.50–0.95; P = 0.02). Among patients with EES, age ≥18, nonwhite race, and elevated baseline erythrocyte sedimentation rate were independently associated with inferior EFS. Conclusion: Clinical characteristics, but not key tumor genomic features, differ between EES and skeletal ES. Extraskeletal origin is a favorable prognostic factor, independent of age, race, and primary site.

Original languageEnglish (US)
Pages (from-to)1771-1779
Number of pages9
JournalPediatric Blood and Cancer
Volume63
Issue number10
DOIs
StatePublished - Oct 1 2016

Keywords

  • extraosseous
  • extraskeletal Ewing sarcoma
  • gene expression
  • gene profiling
  • prognosis
  • soft-tissue Ewing sarcoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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    Cash, T., McIlvaine, E., Krailo, M. D., Lessnick, S. L., Lawlor, E. R., Laack, N., Sorger, J., Marina, N., Grier, H. E., Granowetter, L., Womer, R. B., & DuBois, S. G. (2016). Comparison of clinical features and outcomes in patients with extraskeletal versus skeletal localized Ewing sarcoma: A report from the Children's Oncology Group. Pediatric Blood and Cancer, 63(10), 1771-1779. https://doi.org/10.1002/pbc.26096