Ewing's Sarcoma of the Head and Neck: A Systematic Review

OBJECTIVE:The aim of the present study was to conduct a systematic review of head and neck Ewing sarcoma (ES) concerning patients' demographic and clinical features, histopathological findings, treatment, follow-up, and survival rate. MATERIALS AND METHODS:An electronic search was undertaken in four databases. Articles describing case reports or case series were included. Outcomes were evaluated by the Kaplan-Meier method along with Cox regression. RESULTS:The search yielded 186 studies describing 227 ES cases. The mean age was 22.7 years, and males were slightly more affected. Interestingly, more than half the cases were diagnosed up to 20 years. The respiratory tract was the most reported site, followed by the jawbones. Clinically, symptomatic swelling or nodules were described, with a mean duration of 4 months. Management involved multimodal treatment regimens. Local recurrence, lymph node and distant metastasis were observed in 10.7%, 12.6%, and 20.3% of cases, respectively. Statistical analysis revealed that older patients with distant metastasis had a lower overall survival rate (p < 0.05). CONCLUSION:This study provides an overall view of head and neck ES that can assist oral and maxillofacial pathologists with the diagnosis and extend the knowledge of surgeons and oncologists about this condition.