Background: Ewing sarcoma (ES) is a rare aggressive malignancy of bone and soft tissue that accounts for approximately 3% of all pediatric malignancies. Although ES can affect any bone, it primary presents in long bones such as the humerus, tibia, and femur. The incidence of ES varies significantly with age but is most seen in adolescent patients; although, specific incidence rates in literature vary. The most recent ES incidence estimates come from Jawad et al. (2009) who reported incidence as 0.3-0.4 and 0.9-1.0 per 100,000 in ages 0-9 years and 10-19 years, respectively, based on 1973-2005 data. Gender-specific ES incidence and prevalence has not been reported previously in the published literature.
Objectives: The objective of the analysis was to determine gender- and age-specific ES incidence and prevalence in the pediatric population in the United States.
Methods: Data from the United States (US) Surveillance, Epidemiology, and End Results (SEER) 21 Registries, Nov. 2020 (2000-2018) was analyzed using SEER*Stat software (v8.3.9) to calculate the incidence and prevalence of ES (overall, by gender, and age groups) for 2018. These rates were applied to the age- and gender-specific 2020 US population (from 2020 US Census Bureau population reports) to estimate the burden of pediatric ES in the US.
Results: The estimated overall 2018 incidence rates of ES for age groups < 1 year, 01-04 years, 05-09 years, 10-14 years, and 15-19 years were 0.22, 0.14, 0.37, 0.44, and 0.47 per 100,000, respectively. Incidence was higher among females for ages < 1-09 years. Incidence of ES then increases among males between the ages of 10 and 19 years. The estimated annual incidence in 2020 among males for ages < 1 year, 01-04 years, 05-09 years, 10-14 years, and 15-19 years were 3, 8, 28, 61 and 60, respectively. Incident cases for females ages < 1 year, 01-04 years, 05-09 years, 10-14 years, and 15-19 years were 5, 14, 47, 35 and 42, respectively. The estimated 1-year prevalence among males for ages < 1 year, 01-04 years, 05-09 years, 10-14 years, and 15-19 years were 0, 10, 21, 37, and 59, respectively. For females, the 1-year prevalence of ES among ages < 1 year, 01-04 years, 05-09 years, 10-14 years, and 15-19 years were 0, 0, 7, 41, and 37, respectively. 5-year prevalence for males in the same age groups were 0, 20, 106, 203, and 280 while for females in the same age groups were 0, 24, 73, 136, and 222.
Conclusions: Pediatric ES is a rare disease with increasing incidence in older adolescent age groups compared to children. Population-based studies are needed to better characterize pediatric ES patient population for improving patient care and survival in the US.
