Introduction: Extraskeletal Ewing’s sarcoma (EES) is a rare malignant soft tissue tumor. Those aggressive mesenchymal tumors are characterized by genetic alterations of the Ewing sarcoma gene (EWS) on chromosome 22. Signs and symptoms are usually non-specific. The clinical diagnosis of EES is challenging. Case Presentation: A 31-year-old female patient presented to our hospital with rectal bleeding. Physical examination revealed a painful and erythematous bleeding rectal mass. A pelvic MRI revealed a right-sided well-circumscribed perianal mass measuring 6.8 × 6.0 cm. Surgical excision of the rectal mass was subsequently performed. The resected specimen, including the rectal mass with a pedicle from the posterior rectal wall was sent for histopathological examination. The postoperative period was uneventful. Histopathology examination was suggestive of EES of the rectum. Clinical Discussion: EES is an unusual entity of Ewing's Sarcoma Family of Tumors, which are characterized by pathognomonic translocations. The clinical features of EES include localized pain and/or swelling. Diagnosis of EES relies on histopathology and immunohistochemistry analysis. Imaging modalities such as CT, MRI, and PET/CT are crucial to evaluate EES metastasis and assess local tumor resectability. Conclusion: Due to the rare entity of EES of the rectum, we report the case of a 31-year-old female with rectal bleeding, found to be rectal Ewing’s sarcoma.
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