Population-Based Study of Pediatric Skull Osteosarcoma and Ewing Sarcoma Outcomes

Overview

This study analyzed 124 pediatric and adolescent patients with osteosarcoma (OSC) or Ewing sarcoma (EWS) of the skull using SEER data from 2000 to 2019. Key findings include better survival in younger patients, those with localized disease, and those with EWS compared to OSC. Age, tumor histology, and disease stage independently predicted overall survival.

Background

Primary malignant skull tumors such as osteosarcoma and Ewing sarcoma are rare in children and adolescents, with most prior data limited to case reports. Osteosarcoma commonly affects long bones, making skull involvement unusual, while Ewing sarcoma typically targets pelvis and long bones, with cranial cases being uncommon. The complex skull anatomy near vital structures complicates diagnosis and treatment, and population-based epidemiological data on these tumors in pediatric patients are scarce. This study leverages the SEER database to provide comprehensive insights into the demographics, treatment patterns, and survival outcomes of these rare skull malignancies.

Data Highlights

Key Findings

• Patients aged 0–14 years had significantly better overall survival than those aged 15–19 years (P = 0.003).
• Ewing sarcoma patients demonstrated higher survival rates compared to osteosarcoma patients (P = 0.004).
• Localized disease at diagnosis was associated with significantly improved survival compared to distant metastases (P = 0.003).
• Multivariable Cox regression identified older age (HR 2.58), distant stage (HR 4.11), and osteosarcoma histology (HR 2.78 relative to EWS) as independent predictors of worse overall survival.
• Surgical resection and chemotherapy were commonly utilized treatments, with radiotherapy more frequently administered in Ewing sarcoma cases.

Clinical Implications

Early diagnosis and treatment of skull osteosarcoma and Ewing sarcoma in pediatric patients are critical, as localized disease and younger age are associated with better survival outcomes. Differentiating between histological subtypes is important for prognosis and may guide therapeutic decisions. Multimodal treatment including surgery and chemotherapy remains the cornerstone of management, with radiotherapy playing a larger role in Ewing sarcoma cases.

Conclusion

This population-based analysis highlights the prognostic significance of age, tumor histology, and disease stage in pediatric skull osteosarcoma and Ewing sarcoma. These findings provide valuable epidemiological insights to inform clinical management and future research in these rare malignancies.

References

1. SEER Program, National Cancer Institute -- Surveillance, Epidemiology, and End Results Database