Sublingual Recurring Extra osseous Ewing Sarcoma: A Rare Case Report and Review of Literature

Abstract

Background: Extera skeletal (Extraosseous) Ewing sarcoma is a rare primary tumor of the soft tissue, with an extremely high mortality rate and a bad prognosis which primarily affects young individuals. It occurs without particular clinical signs and can impact several different areas, which may delay diagnosis. Histopathological examination and immunohistochemistry are crucial for making a definite diagnosis. Besides this, early diagnosis is necessary for succeeding of the management. Case report: The objective of the present case report was to document a rare case of EES which originated from the floor of the mouth. A 37-year-old male patient came to our clinic with sublingual rapidly growing swelling. He was suffering from difficulty in opening his mouth and a feeling of dysphagia. The patient revealed during a history-taking procedure that he had undergone one surgery to remove the mass that had previously developed in the same area, knowing that he had been diagnosed in the first as a non-specific inflammatory reaction. Clinical examinations revealed a dome shape soft tissue mass. The biopsy specimen underwent histopathological and immunohistochemical analysis, which demonstrated poorly differentiated small blue round cells with positive immuno-expression of CD-99, NKX2-2, FLI-1, and Vimentin. The final diagnosis was EES based on the clinical presentation, histopathological pattern, and findings of the immunohistochemistry investigations. Radiotherapy and chemotherapy were chosen as treatment modalities for the case. Unfortunately, the patient died after three months due to the occurrence of multiple distant metastasis areas. Conclusion: Regardless of the relative rarity of Extraosseous Ewing sarcoma, it should be taken into account as a possible differential diagnosis for intraoral soft tissue masses, and accurate diagnosis with precise histopathological and immunohistochemical evaluations should be done as soon as possible. Moreover, improvement of management procedures of such cases generally provides the best chances for patient survival.