Ewing sarcoma of the head and neck region in pediatric patients: A retrospective monoinstitutional series

Ewing sarcoma (ES) occurs in the head and neck (HN) region in fewer than 10% of cases, presenting distinct therapeutic challenges.

We retrospectively reviewed all ES cases diagnosed at our institution from 2000 to 2022.

Of 131 patients, 10 (7.6%) had primary HN tumors (median age: 10 years). Tumor locations included the paranasal sinuses (n=6), jaw (n=2), and skull (n=2). Three patients presented with metastatic disease.

All patients received first-line chemotherapy, except one who underwent upfront surgery. Local treatment included radiotherapy (RT) alone (n=4), surgery plus RT (n=4), and surgery alone (n=2). Resection was feasible in six cases, with five patients achieving R0 margins following delayed surgery after Busulfan-Melphalan (BuMel) consolidation. Reconstruction used autologous bone grafts (n=3) or free fibular flaps (n=2), with one graft failure.

One patient had local recurrence at 24 months; three with initial metastases had distant relapse at 8, 29, and 44 months. Median event-free survival (EFS) was 27 months (range: 8–44), and median overall survival (OS) was 63 months (range: 12–159). At last follow-up, five patients were in complete remission, one was receiving third-line therapy, one was lost to follow-up, and three died in disease progression.

In conclusion, metastatic disease at diagnosis remains the strongest predictor of poor outcome. Nevertheless, our findings highlight the importance of a multidisciplinary team approach, with close collaboration among radiation oncologists and maxillofacial surgeons. Such integrated teamwork may improve prognosis and help reduce long-term sequelae.