Primary Ewing's sarcoma of the ethmoid sinus with intracranial and orbital extension: case report and literature review

Abstract

The Ewing's sarcoma family of tumors is a group of cancers that commonly arises in young adults during their second decade of life. It frequently involves the trunk and long bones of the body with primary Ewing's sarcoma of the paranasal sinuses being exceedingly rare. We describe the case of a 39 year-old female with primary Ewing's Sarcoma originating from the ethmoid sinus with intracranial extension into the anterior cranial fossa and the orbit. The radiologic and histopathologic profiles are presented with a review of the literature. To our knowledge, this is the second reported case with the tumor involving the anterior cranial fossa, but the only case where immunochemical staining and molecular genetic analysis are available for definitive diagnosis.