Osteosarcoma and Ewing sarcoma of the skull in children and adolescents: a population-based study.

PURPOSE

Osteosarcoma (OSC) and Ewing sarcoma (EWS) of the skull are rare malignancies in children and adolescents. This study aimed to analyze the demographic, clinical, treatment, and survival characteristics of these tumors using a population-based approach.

METHODS

Data from the Surveillance, Epidemiology, and End Results (SEER) database were used to identify pediatric and adolescent patients (aged 0-19 years) diagnosed with OSC or EWS of the skull from 2000 to 2019. Demographic, clinical, and treatment variables were analyzed. Kaplan-Meier survival analysis and Cox proportional hazards regression models were used to evaluate prognostic factors for overall survival (OS).

RESULTS

A total of 124 patients were included, with 46% diagnosed with OSC and 54% with EWS. At diagnosis, 43.6% had localized disease, 42.6% had regional disease, and 13.8% presented with distant metastases. Surgical resection was the primary treatment (84.6%), with 84.6% of patients receiving chemotherapy and 43.6% undergoing radiotherapy. The OS rates for the entire cohort were 90.3% at 1 year, 77.9% at 3 years, and 75.6% at 5 years. Younger patients (0-14 years) demonstrated significantly better survival outcomes compared to older adolescents (P = 0.003). EWS was associated with higher survival rates than OSC (P = 0.004). Patients with localized disease had significantly better survival outcomes compared to those with distant metastases (P = 0.003). Multivariable Cox regression analysis identified age, histological subtype, and SEER stage as independent prognostic factors. Older patients had higher mortality risk (HR 2.58; 95% CI, 1.28-5.19; P = 0.008), as did those with distant-stage disease (HR 4.11; 95% CI, 1.67-10.11; P = 0.002). EWS was associated with better survival compared to OSC (HR 0.36; 95% CI, 0.13-0.99; P = 0.048).

CONCLUSIONS

This study highlights significant differences in survival outcomes for OSC and EWS of the skull in children and adolescents. Younger age, localized disease, and EWS subtype were associated with better prognosis. These findings underscore the need for early diagnosis and tailored treatment strategies to improve outcomes in this population.