A Rare Case of Extraosseous Ewing’s Sarcoma/Primitive Neuroectodermal Tumor in Female

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. Diagnosis typically involves imaging studies such as magnetic resonance imaging (MRI), computed tomography (CT) scans, and biopsy for confirmation. Treatment typically includes a combination of chemotherapy, surgery to remove the tumor, and sometimes radiation therapy. In this report, we present a case of EES in a 44-year-old female who presented with complaints of swelling in the right thigh for 3 months. The mass was soft in consistency with well-delineated borders, evident both clinically and radiologically, extending into the subcutaneous layer and involving the right inguinal lymph nodes. The mass was widely excised with en-bloc dissection of the right inguinal nodes. The histopathological features confirmed the diagnosis and have been discussed in this study. This case underscores the clinical significance of EES, a rare variant presenting diagnostic challenges. Our findings highlight the importance of prompt diagnosis and early management to improve outcomes for patients with this aggressive malignancy.