Pediatric head and neck bone sarcomas: An analysis of 204 cases.

OBJECTIVES: To analyze the demographics, survival, and treatment efficacy of pediatric sarcomas of the facial skeleton and skull.

METHODS: Retrospective study of cases from the US National Cancer Institute's Surveillance, Epidemiology, and End Results database. Pediatric patients between the ages of 0 and 18 diagnosed with a malignant sarcoma of either the mandible or the bones of skull, face, and associated joints from 1973 to 2013 were studied.

RESULTS: In total, 204 patients were included in the analysis. The average age at diagnosis was 11.39 (±5.15) years with a male-to-female ratio of 1.4:1. Whites were the most commonly affected race (76.0%). Malignant mandible sarcomas accounted for 29.9% of the cohort (n = 61). The most common pathology was osteosarcoma, which accounted for 43.6% of the cohort (n = 89). Among patients with known histologic grade (n = 95), 26.0% were AJCC stage III or IV. Overall, 5-year disease-specific survival (DSS) was 80.6%. When stratified by treatment modality, 5-year DSS was 86.0% for surgery alone, 67.9% for radiation alone, and 75.3% for surgery with adjuvant radiotherapy (p = 0.041).

CONCLUSIONS: Osteosarcoma, Ewing's sarcoma, and chondrosarcoma are the most common subtypes of pediatric head and neck bone sarcoma. Such sarcomas more commonly affect whites and males during pubertal ages. Disease-specific survival is not affected by primary site. Surgery alone is the mainstay of treatment, and demonstrates higher 5-year disease-specific survival compared to radiotherapy alone. Adjuvant radiotherapy does not seem to increase survival, but further investigation is warranted.