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Ewing sarcoma family tumors (ESFT) pose diagnostic challenges, which largely depend on the primary site of involvement and tumor stage. Despite advancements in treatment, metastatic ESFTs remain associated with poor outcomes. This case describes a 21-year-old woman who, in July 2022, presented with a left breast mass identified through ultrasound and CT scan, along with abdominal distention.

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Background: Primary intracranial Ewing Sarcoma/peripheral Primitive Neuroectodermal Tumor (EWS/pPNET) is exceedingly rare and easy to misdiagnose.

Case Presentation: We present a case involving a 23-year-old male who presented with headaches and vomiting. The preoperative brain imaging revealed an irregular mass in the left parietal lobe, initially misdiagnosed as meningioma.

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Second Department of Pathology, Medical School, National and Kapodistrian University of Athens, Attikon University Hospital, 15772 Athens, Greece.

Ewing sarcomas are rare tumors arising mainly in the bones and the surrounding soft tissues. Primary extraosseous Ewing sarcomas have also been described in several other organs and locations other than bones, including the pancreas. These tumors have well-defined histological, immunohistochemical, and molecular characteristics.

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Extraosseous Ewing's sarcoma (EES) is a rare form of Ewing's sarcoma that arises outside the bones in soft-tissue structures. It is thought to result from a genetic abnormality involving the fusion of the EWSR1 gene with various partner genes, most commonly the FLI-1 gene. Common symptoms include pain, swelling, and sometimes a palpable mass at the site of the tumor.

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