Primary Ewing sarcoma of the sphenoid bone with infrasellar spread in a 17-year-old patient: case report and literature review

Abstract

We performed retrospective analysis of publications describing cases of Ewing sarcoma of the skull base with exceedingly rare locations (including the area of the sphenoid bone, its wings, sinus, and adjacent structures). Ewing sarcoma is usually diagnosed in children. The peak incidence is registered in patients aged 10–15 years, whereas adults and children under 5 years of age are very unlikely to develop this disease. In this article, we also report a case of Ewing sarcoma of the skull bones affecting the sphenoid bone and sphenoid sinus. The patient has undergone surgery followed by chemoradiotherapy. High invasion potential (tumor spread in several directions), high malignancy, and compression of the adjacent structures of the brain make the analysis of Ewing sarcoma located in areas that cannot be easily approached particularly interesting for neurosurgeons, since surgery is the main treatment option for such patients.