Endoscopic endonasal management of extraskeletal sinonasal ewing’s sarcoma

Abstract

Ewing’s sarcoma (ES) is a primary neoplasm of the skeletal system. Ewing’s sarcoma of the head-and-neck region constitutes about 1–4% of extraskeletal Ewing’s sarcoma (EES cases). Sinonasal EES is extremely rare and there is limited literature on this entity. Establishing the diagnosis requires a histopathological examination, immunohistochemistry, and a cytogenetic analysis along with a CT scan and MRI of the paranasal sinuses. They require multimodality treatment including surgical resection followed by chemotherapy and/or radiotherapy. We present a case report of a 31-year-old male patient presenting with a right-sided headache associated with nausea and vomiting, right nasal obstruction, a mass protruding from the right nostril and associated with occasional nasal bleed for 4 months with aggravation of symptoms since last 15 days along with proptosis of the right eye. On nasal examination, reddish-brown friable slough covered mass protruding from the right nostril bleeding on manipulation. Endoscopic endonasal excision biopsy revealed a round cell tumor-ES/primitive neuroectodermal tumor (PNET). The patient received radiotherapy and chemotherapy postoperatively and is on regular follow-up for 2 years and is disease-free.