Combined modality treatment of Ewing's sarcoma of the maxilla.

Overview

BACKGROUND: Primary bone sarcomas arising in osseous structures of the head and neck are rare. These tumors are often incompletely resected and treated with radiotherapy for local control. METHODS: We report a case of a 9-year-old girl with a maxillary Ewing's sarcoma. This patient was successfully treated with neoadjuvant chemotherapy followed by surgical resection and prosthetic reconstruction of the primary site. The surgical approach that was used consisted of a subtotal maxillectomy by means of a facial degloving approach, sparing the orbital contents and the inferior orbital rim and orbital floor. RESULTS: This approach produced excellent cosmetic, functional, and oncologic outcome. The patient remains without evidence of disease recurrence more than 4 years after surgery. CONCLUSIONS: This case illustrates a novel surgical approach to the resection of a maxillary Ewing's sarcoma and highlights the need for a multidisciplinary team approach to the management of head and neck sarcomas in children.