Ewing's sarcoma of the maxillofacial region in Greek children: Report of 6 cases and literature review

Abstract

Background: The rarity of Ewing's sarcoma (ES) in the maxillofacial region of children, coupled with the technical challenge of resection and associated functional and cosmetic impairment has resulted in deficient data regarding the optimal local control of the disease.

Objective: To describe our experience in the management of primary maxillofacial ES in children, focusing on the therapeutic modalities for local control of the disease.

Study design: Single institution observational study.

Methods: This is a single institution review of patients, treated between 2007 and 2016.

Results: Six primary maxillofacial ES were treated according to the EURO-EWING 99 protocol, consisting of a uniform chemotherapy regimen, combined selectively with surgery and radiotherapy as local treatment. Patients' mean age was 9.42 years (range 6-12.5 years). One patient initially suffered from metastasis and succumbed to the disease; another refused further treatment following chemotherapy and was lost to follow-up. Four patients underwent surgery and adjuvant radiotherapy successfully. At a mean follow-up of 3.78 years relapse-free and overall survival rates were 60% and 80% respectively. The aesthetic and functional outcome was satisfactory in all treated patients.

Conclusions: In eligible cases the combination of chemotherapy with surgery and adjuvant radiotherapy results in optimal oncological and functional outcome for children with ES of the maxillofacial region. Metastasis and poor response to chemotherapy are the most important adverse prognostic factors.

Keywords: Children; Ewing's sarcoma (ES); Maxillofacial region; Radiotherapy; Surgical treatment.