Primary peripheral neuroepithelioma of the orbit with intracranial extension

Abstract

A 7-year-old girl with clinical signs limited to moderate unilateral proptosis of 2 weeks duration and ipsilateral disc edema was found to have a contiguous orbital and subfrontal intracranial tumor best characterized as a peripheral neuroepithelioma by recent studies. Previously this tumor would have been called an extraosseous Ewing's sarcoma. The tumor had a significant lobular component on either side of the orbital roof. The patient is still alive 24 months posttreatment with multimodal excisional surgery, radiation, and chemotherapy.