Primary peripheral neuroepithelioma of the orbit with intracranial extension.
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.
Wilson
W B
WB
Department of Ophthalmology, University of Colorado Health Sciences Center, Denver.
Roloff
J
J
Wilson
H L
HL
D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D009918:Orbital Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D012512:Sarcoma, Ewing / Q000473:pathology; D014057:Tomography, X-Ray Computed