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Clinical features and outcomes of infants with Ewing sarcoma under 12 months of age
Thalia Wong1, Robert E Goldsby1, Rosanna Wustrack2
1Department of Pediatrics, San Francisco School of Medicine, UCSF Benioff Children's Hospital, University of California, San Francisco, California.
Insights
Ewing sarcoma is rare in infants, presenting differently and treated less aggressively. While infants face higher early death risks, their long-term survival for this rare cancer is similar to older children.
Area of Science:
- Pediatric Oncology
- Cancer Epidemiology
- Rare Pediatric Cancers
Background:
- Ewing sarcoma incidence peaks in adolescence, with rare reports in infants under 12 months.
- Understanding clinical characteristics and outcomes in infant Ewing sarcoma is crucial due to its rarity.
Purpose of the Study:
- To compare clinical features, treatment modalities, and survival rates of infants (<12 months) with Ewing sarcoma against older pediatric patients (1-19 years).
Main Methods:
- Utilized the Surveillance, Epidemiology, and End Results (SEER) database (1973-2011).
- Compared clinical features and treatment using Fisher exact tests.
- Assessed overall survival via Kaplan-Meier methods.
Main Results:
- Infants (2.0% of cohort) presented with more soft tissue tumors and primitive neuroectodermal tumors/Askin tumors, and smaller tumor size (<8 cm).
- Infants received radiation therapy less frequently (13.2% vs. 53.3%).
- Infants showed increased risk for early death, but similar long-term overall survival compared to older patients.
Conclusions:
- Ewing sarcoma in infants (<12 months) exhibits distinct clinical presentations and treatment patterns.
- Infants with Ewing sarcoma have a higher risk of early mortality but comparable long-term survival to older pediatric patients.
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.
