Clinical features and outcomes of infants with Ewing sarcoma under 12 months of age

Pediatr Blood Cancer. 2015 Nov;62(11):1947-51. doi: 10.1002/pbc.25635. Epub 2015 Jul 14.

Abstract

Background: Ewing sarcoma peaks in incidence in adolescence. Infants <12 months old have rarely been reported. We aimed to compare clinical features, treatment, and survival of infants <12 months to those of older pediatric patients with Ewing sarcoma.

Procedure: We utilized the SEER database to identify patients <12 months of age diagnosed with Ewing sarcoma between 1973 and 2011. We used Fisher exact tests to compare clinical features and treatment modalities between these patients and patients aged 1-19 years. We used Kaplan-Meier methods to describe overall survival in these two groups.

Results: Of 1,957 patients in the cohort, 39 (2.0%) were diagnosed at <12 months of age. Infants had a different distribution of primary tumor sites, with lower extremity tumors under represented. Compared to older patients, infants were more likely to have soft tissue tumors (81.6% vs. 27.1%; P < 0.001); have primitive neuroectodermal tumor/Askin tumor (61.5% vs. 19.9%; P < 0.001); and have tumors <8 cm (81.0% vs. 53.2%; P < 0.014). Infants were less likely to receive radiation therapy (13.2% vs. 53.3%; P < 0.001). Infants were at increased risk for early death (P < 0.013 by Wilcoxon), though long-term overall survival was not different between age groups (P < 0.25 by log rank).

Conclusions: Ewing sarcoma is rare in infants, with different clinical presentations and treatment approaches. These patients appear to be at higher risk for early death, but long-term survival is similar to older pediatric patients.

Keywords: Ewing sarcoma; infants; local control; survival.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Databases, Factual*
  • Disease-Free Survival
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Retrospective Studies
  • Risk Factors
  • Sarcoma, Ewing / mortality*
  • Sarcoma, Ewing / pathology*
  • Survival Rate
  • United States / epidemiology