title: Primitive neuroectodermal tumor/Ewing sarcoma of the retina.
abstract: An 11-year-old boy underwent enucleation of his left eye for an intraocular tumor. Examination showed a small, round blue cell tumor arising in the peripheral retina near the ciliary body. Immunohistochemical stain results were positive for neuron-specific enolase, synaptophysin, cluster of differentiation 99 (CD99), Friend leukemia integration 1, and CD56. Ultrastructural findings included occasional intracytoplasmic dense core granules. Polymerase chain reaction of the tumor showed a Ewing sarcoma/Friend leukemia integration gene fusion product. The tumor was classified as a primitive neuroectodermal tumor/Ewing sarcoma of the retina and should be distinguished from retinoblastoma. To our knowledge, this is the first case of primary primitive neuroectodermal tumor of the retina.
authors: Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia 30322, USA. ophtheg@emory.edu
authors: Hans E
authors: HE
authors: Grossniklaus
authors: Bahig
authors: B
authors: Shehata
authors: Poul
authors: P
authors: Sorensen
authors: Chris
authors: C
authors: Bergstrom
authors: G Baker
authors: GB
authors: Hubbard
mesh_terms: D002648:Child; D006801:Humans; D008297:Male; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D015514:Oncogene Proteins, Fusion / Q000235:genetics / Q000378:metabolism; D010751:Phosphopyruvate Hydratase / Q000235:genetics / Q000378:metabolism; D051789:Proto-Oncogene Protein c-fli-1 / Q000235:genetics / Q000378:metabolism; D019572:Retinal Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D012512:Sarcoma, Ewing / Q000235:genetics / Q000378:metabolism / Q000473:pathology*
chemical_list: D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D010751:Phosphopyruvate Hydratase