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

Abstract

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. 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. This approach produced excellent cosmetic, functional, and oncologic outcome. The patient remains without evidence of disease recurrence more than 4 years after surgery. 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.