Atypical presentation of extra-skeletal Ewing’s sarcoma in a 57-year-old female – a case report

Abstract

Introduction and aim. Malignant soft tissue tumors exhibiting similar histological and immunohistochemical characteristics to Ewing sarcoma of the bones are referred to as extra-skeletal Ewing sarcoma within the pathology research domain. These tumors fall under the broader classification of Ewing sarcoma family of tumors, which encompasses Ewing sarcoma of the bones, extra-skeletal Ewing sarcoma, and primitive neuroectodermal tumor, the latter demonstrating a more pronounced neural differentiation compared to Ewing sarcoma of the bone. Extra-skeletal Ewing sarcoma stands out as a rare, aggressive, and rapidly growing malignant soft tissue tumor characterized by a notable recurrence rate and a predilection for occurrence in males. The roots of recognizing extra-skeletal Ewing sarcoma trace back to 1975 when Angervall and Enzinger reported the inaugural case. This study aims to underscore the significance of recognizing diverse clinical presentations for precise diagnosis and effective patient care of Extra-skeletal Ewing sarcoma in an elderly patient.

Case description. In the context of our pathology research, a noteworthy case involves a 57-year-old female presenting with a mass in the left iliac fossa. The diagnosis, established through a comprehensive approach involving Imaging, histopathological examination, immunohistochemistry, and molecular studies such as fluorescence in situ hybridization, confirms the nature of the tumor as extra-skeletal Ewing sarcoma. This case adds to the understanding and documentation of this distinct variant through a multi-modal investigative process.

Conclusion. This case report contributes to the existing literature by shedding light on an atypical presentation of extra-skeletal Ewing sarcoma in an older patient. Understanding the varied clinical manifestations and incorporating advanced diagnostic techniques, such as fluorescence in situ hybridization and immunohistochemistry, is pivotal for accurate diagnosis and optimal patient management.