Abstract
The Ewing sarcoma family of tumors (ESFT) is the second most common primary pediatric tumor. However, ESFT of the retromolar trigone (RMT) is rare and this article reports such a case in a 10-year-old boy who presented with right-sided facial swelling. His computerized tomography (CT) scan revealed a lesion involving the right RMT region with no evidence of distant metastasis. Histopathological examination revealed a poorly differentiated malignant mass and immunohistochemistry confirmed the diagnosis of ESFT with a strong FLI1 positivity. He was started on a high-risk protocol followed by definitive radiation therapy of 54 Gy/30# by intensity-modulated radiation therapy technique. The patient showed complete response to the treatment with positron emission tomography-CT showing no evidence of active disease.
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