Ewing sarcoma family of tumors of the retromolar trigone

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. (author)