Ewing's sarcoma of the head and neck in children

Objective: The purpose of this paper was to review our experience with Ewing's sarcoma of the head and neck in children. Design: Retrospective chart review. Setting: The Hospital for Sick Children, Toronto, Ont., Canada. Methods: Between 1986 and 1996, 70 cases of Ewing's sarcoma were identified. The medical records, roentgenographic and pathology reports were reviewed retrospectively. The gender, age of presentation, location and clinical presentation of the tumor were noted in the cases involving the head and neck. The treatment and follow-up of these patients were recorded. Results: Of the 70 cases of Ewing's, five involved the head and neck (7.1%). The age of presentation ranged from 7.5 to 14 years. An enlarging mass in the mandible was the mode of presentation in three of the five children. Two patients had metastases at initial presentation. All patients received combination treatment regimens with chemotherapy initially, followed by adjuvant surgery and/or radiation. Follow-up ranged from 2 to 11 years. Three of five patients died of metastatic disease. Two are alive and well with no evidence of disease. Conclusions: Ewing's sarcoma occurs infrequently in the head and neck in children. An enlarging mass in the mandible is the most Frequent mode of presentation. This tumor is treated systemically with high dose chemotherapy and locally with surgical excision where possible. In lesions that are initially unresectable and:or show a poor response to chemotherapy, radiation is used for local control. A good prognosis can be expected if the disease has not metastasized. (C) 1999 Elsevier Science Ireland Ltd. All rights reserved.