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.

This publication has 16 references indexed in Scilit: