Aggressive primary sinonasal Ewing sarcoma with intraorbital and intracranial extension

Abstract

Ewing sarcoma (ES) of the extraskeletal form is a rare occurrence and even rarer to manifest in the sinonasal tract. We describe a case of an advanced sinonasal ES with orbital and intracranial extension that was managed with chemotherapy and endoscopic surgery. Despite completing chemotherapy and surgical resection, the tumor progressed rapidly even before postoperative radiotherapy, and subsequently, she succumbed to death due to sepsis. We would like to discuss the management challenges, pitfalls, and surgical approach which may improve the outcome of this complicated disease.