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  • Title: Primitive neuroectodermal tumor of the orbit in an adult. A case report and literature review.
    Author: Kiratli H, Bilgiĉ S, Gedikoğlu G, Ruacan S, Ozmert E.
    Journal: Ophthalmology; 1999 Jan; 106(1):98-102. PubMed ID: 9917788.
    Abstract:
    OBJECTIVE: The authors describe the salient clinical, radiologic, and histopathologic features of an orbital primitive neuroectodermal tumor in a 28-year-old man. This is an extremely rare tumor of the orbit, previously reported exclusively in children. DESIGN: Case report. INTERVENTION: Excisional biopsy of the tumor en bloc was performed. MAIN OUTCOME MEASURES: Histopathologic examination was performed by standard techniques and immunohistochemical stains on formaldehyde-fixed, paraffin-embedded tumor tissues. RESULTS: Histologic examination of the sections of the tumor showed small, blue, round cells with occasional Homer-Wright rosette formations. The tumor cells stained positively with neuron-specific enolase and vimentin. CONCLUSIONS: This newly recognized, highly unusual peripheral primitive neuroectodermal tumor should be considered in the differential diagnosis of hypercellular, small, round cell tumor of the orbit in adults.
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