[Ewing's sarcoma of the jaw. The value of multidisciplinary management. Apropos of 4 cases]

Abstract

Ewing's sarcoma is the second most common malignant bone tumor of childhood, yet it is a rare tumor. Primary maxillary localization is unusual and occur in only 1-2% of cases, mostly in the mandible. The prognosis of Ewing's sarcoma has been improving considerably since the introduction of combined modality treatment. The estimated overall survival at 4 years is 75%. It is widely accepted that prompt chemotherapy is necessary to treat occult micrometastasis, present in over 80% of cases at time of diagnosis, and to reduce the tumor size. Prognostic factors correlated with a poor overall survival are large tumor size and poor histologic response to initial chemotherapy. Adequate local control of Ewing's sarcoma can be achieved after chemotherapy, with radical or conservative surgery and radiotherapy. Surgery was recommended whenever possible. Radiation dosage and fields are based upon the quality of surgery and histological response to chemotherapy. Concern has been raised, however, regarding deleterious late effects of radiation in this young population. Conservative surgery and reconstruction are often used to improve functional outcome. We report four cases of Ewing's sarcoma localized to jaw bone and mandible, successfully treated by combined modality treatment.