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Ewing's sarcoma of the head and neck in children
J P Vaccani1, V Forte, A L de Jong
1Department of Otolaryngology, The Hospital for Sick Children, University of Toronto, Ont., Canada.
Insights
Ewing's sarcoma of the head and neck is rare in children, often presenting as a mandibular mass. Early detection and treatment with chemotherapy, surgery, and radiation improve outcomes, especially if the disease has not spread.
Area of Science:
- Pediatric Oncology
- Head and Neck Surgery
- Sarcoma Research
Background:
- Ewing's sarcoma is a rare bone and soft tissue cancer.
- Head and neck involvement in pediatric Ewing's sarcoma is infrequent.
Purpose of the Study:
- To review the experience with pediatric head and neck Ewing's sarcoma.
- To analyze presentation, treatment, and outcomes.
Main Methods:
- Retrospective chart review of 70 Ewing's sarcoma cases (1986-1996).
- Focused analysis on 5 head and neck cases, noting demographics, clinical presentation, treatment, and follow-up.
Main Results:
- Five cases (7.1%) of head and neck Ewing's sarcoma identified in children aged 7.5-14 years.
- Mandibular mass was the most common presentation (3/5); 2 patients had metastases.
- All received chemotherapy, surgery, and/or radiation; 3/5 died of metastatic disease, 2 are alive with no evidence of disease.
Conclusions:
- Pediatric head and neck Ewing's sarcoma is uncommon, frequently presenting as a mandibular mass.
- Treatment involves systemic chemotherapy and local control via surgery and/or radiation.
- Prognosis is favorable for non-metastatic disease.
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.
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