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

Abstract

Introduction: 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.

Case Presentation: In this report we present a case of Extraosseous Ewing’s sarcoma in a 44 year old female who presented with the 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 has been discussed in this study.

Conclusions: This case underscores the clinical significance of extraosseous Ewing's sarcoma, 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.