Sinonasal Ewing Sarcoma: A Case Report and Literature Review.

INTRODUCTION

Ewing sarcoma (ES) of the sinonasal tract and associated primitive neuroectodermal tumors are rare. To our knowledge, only 10 case reports of sinonasal ES of the nose or paranasal sinuses have been reported. Furthermore, there has been only 1 case of sinonasal ES arising from the middle turbinate. Recommended management of sinonasal ES stems from the management of its osseous counterpart, ES, but treatment with surgery, radiotherapy, and chemotherapy is varied. Five-year survival rates vary from 21% to 70%, with the lower rates representing patients presenting with metastatic disease.

CASE PRESENTATION

A 26-year-old man presented with persistent left-sided nasal obstruction. Endoscopy demonstrated a friable mass in the left nasal cavity originating from the middle turbinate with extension into the nasopharynx, confirmed with computed tomography and magnetic resonance imaging. There was no evidence of metastatic disease on positron emission tomography-computed tomography. Histopathologic results were consistent with sinonasal ES. The result of fluorescent in situ hybridization was positive for the EWS gene translocation. A multidisciplinary tumor board evaluated the patient. The patient then underwent neoadjuvant chemotherapy, followed by definitive endoscopic surgical resection and postoperative radiotherapy.

DISCUSSION

Our literature review found more involvement of the maxillary and ethmoid sinuses compared with the nasal cavity, and that the role of radiation and surgical approach was varied. ES of the sinonasal tract is a rare entity with high mortality, but few standardized treatment protocols exist. Further study and evidence-based treatment protocols are needed. This article outlines the role of relevant imaging, a multidisciplinary team approach, and the optimal timing of surgery, chemotherapy, and radiation.