{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,3]],"date-time":"2026-03-03T00:29:08Z","timestamp":1772497748904,"version":"3.50.1"},"reference-count":32,"publisher":"Archives of Pathology and Laboratory Medicine","issue":"7","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2012,7]]},"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.5858\/arpa.2011-0403-cr","type":"journal-article","created":{"date-parts":[[2012,6,28]],"date-time":"2012-06-28T15:53:59Z","timestamp":1340898839000},"page":"829-831","source":"Crossref","is-referenced-by-count":11,"title":["Primitive Neuroectodermal Tumor\/Ewing Sarcoma of the Retina"],"prefix":"10.5858","volume":"136","author":[{"given":"Hans E.","family":"Grossniklaus","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bahig","family":"Shehata","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Poul","family":"Sorensen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chris","family":"Bergstrom","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G. Baker","family":"Hubbard","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"3906","reference":[{"issue":"8","key":"arpa-136-07-829-831-b01","series-title":"Arch Pathol Lab Med","first-page":"1203","article-title":"High-risk features and tumor differentiation in retinoblastoma: a retrospective histopathologic study","volume":"133","author":"Eagle\n              RC\n              Jr","year":"2009","unstructured":"Eagle\n              RC\n              Jr\n            \n          . High-risk features and tumor differentiation in retinoblastoma: a retrospective histopathologic study. Arch Pathol Lab Med. 2009;133(8):1203\u20131209."},{"issue":"11","key":"arpa-136-07-829-831-b02","series-title":"Ophthalmology","first-page":"1565","article-title":"Recurrent medulloepithelioma of the ciliary body: immunohistochemical characteristics","volume":"95","author":"Kivela\n              T,","year":"1988","unstructured":"Kivela\n              T, \n            \n              Tarkkanen\n              A\n            \n          . Recurrent medulloepithelioma of the ciliary body: immunohistochemical characteristics. Ophthalmology. 1988;95(11):1565\u20131575."},{"issue":"2","key":"arpa-136-07-829-831-b03","series-title":"Arch Ophthalmol","first-page":"217","article-title":"Primitive neuroectodermal tumor of the orbit","volume":"112","author":"Singh\n              AD,","year":"1994","unstructured":"Singh\n              AD, \n            \n              Husson\n              M, \n            \n              Shields\n              CL, \n            \n              DePotter\n              P, \n            \n              Shields\n              JA\n            \n          . Primitive neuroectodermal tumor of the orbit. Arch Ophthalmol. 1994;112(2):217\u2013221."},{"issue":"3","key":"arpa-136-07-829-831-b04","series-title":"Cancer","first-page":"725","article-title":"Extraskeletal Ewing's sarcoma","volume":"85","author":"Ahmad\n              R,","year":"1999","unstructured":"Ahmad\n              R, \n            \n              Mayol\n              BR, \n            \n              Davis\n              M\n            , et al. \n          Extraskeletal Ewing's sarcoma. Cancer. 1999;85(3):725\u2013731."},{"issue":"12","key":"arpa-136-07-829-831-b05","series-title":"Pediatr Radiol","first-page":"1365","article-title":"Extraosseous Ewing sarcoma of the thyroid gland","volume":"39","author":"Adapa\n              P,","year":"2009","unstructured":"Adapa\n              P, \n            \n              Chung\n              T, \n            \n              Popek\n              EJ\n            , et al. \n          Extraosseous Ewing sarcoma of the thyroid gland. Pediatr Radiol. 2009(12); 39:1365\u20131368."},{"issue":"4","key":"arpa-136-07-829-831-b06","series-title":"Indian J Radiol Imaging","first-page":"305","article-title":"Case report: intracranial peripheral primitive neuroectodermal tumor\u2014Ewing's sarcoma of dura with transcalvarial-subgaleal extension: an unusual radiological presentation","volume":"19","author":"Bano\n              S,","year":"2009","unstructured":"Bano\n              S, \n            \n              Yadav\n              SN, \n            \n              Garga\n              UC\n            \n          . Case report: intracranial peripheral primitive neuroectodermal tumor\u2014Ewing's sarcoma of dura with transcalvarial-subgaleal extension: an unusual radiological presentation. Indian J Radiol Imaging. 2009;19(4):305\u2013307."},{"issue":"2","key":"arpa-136-07-829-831-b07","series-title":"Indian J Radiol Imaging","first-page":"151","article-title":"Extraosseous Ewing's sarcoma\/primitive neuroectodermal tumor of the sacral nerve plexus","volume":"19","author":"Narula\n              MK,","year":"2009","unstructured":"Narula\n              MK, \n            \n              Gupta\n              N, \n            \n              Anand\n              R\n            , et al. \n          Extraosseous Ewing's sarcoma\/primitive neuroectodermal tumor of the sacral nerve plexus. Indian J Radiol Imaging. 2009;19(2)151\u2013154."},{"key":"arpa-136-07-829-831-b08","series-title":"Pathology and Genetics of Tumors of Soft Tissue and Bone","first-page":"298","article-title":"Ewing sarcoma\/primitive neuorectodermal tumour (PNET)","author":"Ushigome\n              S,","year":"2002","unstructured":"Ushigome\n              S, \n            \n              Machinami\n              R, \n            \n              Sorensen\n              PH\n            \n          . Ewing sarcoma\/primitive neuorectodermal tumour (PNET). In: FletcherCD, UnniKK, MertensF, eds. Pathology and Genetics of Tumors of Soft Tissue and Bone. Lyon, France: IARC Press; 2002:298\u2013300. World Health Organization Classification of Tumours; vol."},{"issue":"4","key":"arpa-136-07-829-831-b09","series-title":"Pediatr Pathol","first-page":"387","article-title":"Peripheral neurogenic tumors of the soft tissues in children and adolescents: a clinicopathologic study of 139 cases","volume":"9","author":"Coffin\n              CM,","year":"1989","unstructured":"Coffin\n              CM, \n            \n              Dehner\n              LP\n            \n          . Peripheral neurogenic tumors of the soft tissues in children and adolescents: a clinicopathologic study of 139 cases. 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Am J Pathol. 1987;127(2):288\u2013304."},{"issue":"5","key":"arpa-136-07-829-831-b12","series-title":"Virchows Arch A Pathol Anat Histopathol","first-page":"421","article-title":"Malignant peripheral neuorectodermal tumors of bone other than Askin's neoplasm: characterization of 14 new cases with immunohistochemistry and electron microscopy","volume":"412","author":"Llombart-Bosch\n              A,","year":"1988","unstructured":"Llombart-Bosch\n              A, \n            \n              Lacombe\n              MJ, \n            \n              Peydro-Olaya\n              A\n            , et al. \n          Malignant peripheral neuorectodermal tumors of bone other than Askin's neoplasm: characterization of 14 new cases with immunohistochemistry and electron microscopy. Virchows Arch A Pathol Anat Histopathol. 1988;412(5):421\u2013430."},{"issue":"11","key":"arpa-136-07-829-831-b13","series-title":"Mod Pathol","first-page":"1175","article-title":"Differentiating lymphoblastic lymphoma from Ewing's sarcoma: lymphocyte markers and gene rearrangement","volume":"14","author":"Ozdemirli\n              M,","year":"2001","unstructured":"Ozdemirli\n              M, \n            \n              Fanburg-Smith\n              JC, \n            \n              Hartmann\n              DP\n            , et al. \n          Differentiating lymphoblastic lymphoma from Ewing's sarcoma: lymphocyte markers and gene rearrangement. Mod Pathol. 2001;14(11):1175\u20131182."},{"issue":"5","key":"arpa-136-07-829-831-b14","series-title":"N Engl J Med","first-page":"294","article-title":"The Ewing family of tumors\u2014a subgroup of small-round-cell tumors defined by specific chimeric transcripts","volume":"331","author":"Delattre\n              O,","year":"1994","unstructured":"Delattre\n              O, \n            \n              Zucman\n              J, \n            \n              Melot\n              T\n            , et al. \n          The Ewing family of tumors\u2014a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med. 1994;331(5):294\u2013299."},{"issue":"12","key":"arpa-136-07-829-831-b15","series-title":"Am J Surg Pathol","first-page":"1657","article-title":"Immunohistochemical detection of FLI-1 protein expression: a study of 132 round cell tumors with emphasis on CD99-positive mimics of Ewing's sarcoma\/primitive neuorectodermal tumor","volume":"24","author":"Folpe\n              AL,","year":"2000","unstructured":"Folpe\n              AL, \n            \n              Hill\n              CE, \n            \n              Parham\n              DM\n            , et al. \n          Immunohistochemical detection of FLI-1 protein expression: a study of 132 round cell tumors with emphasis on CD99-positive mimics of Ewing's sarcoma\/primitive neuorectodermal tumor. Am J Surg Pathol. 2000;24(12):1657\u20131662."},{"issue":"3","key":"arpa-136-07-829-831-b16","series-title":"Arch Ophthalmol","first-page":"207","article-title":"Metastasis of Ewing's sarcoma to the choroid","volume":"89","author":"Jampol\n              LM,","year":"1973","unstructured":"Jampol\n              LM, \n            \n              Cottle\n              E, \n            \n              Fischer\n              DS\n            , et al. \n          Metastasis of Ewing's sarcoma to the choroid. Arch Ophthalmol. 1973;89(3):207\u2013209."},{"issue":"8","key":"arpa-136-07-829-831-b01","series-title":"Arch Pathol Lab Med","first-page":"1203","article-title":"High-risk features and tumor differentiation in retinoblastoma: a retrospective histopathologic study","volume":"133","author":"Eagle\n              RC\n              Jr","year":"2009","unstructured":"Eagle\n              RC\n              Jr\n            \n          . High-risk features and tumor differentiation in retinoblastoma: a retrospective histopathologic study. Arch Pathol Lab Med. 2009;133(8):1203\u20131209."},{"issue":"11","key":"arpa-136-07-829-831-b02","series-title":"Ophthalmology","first-page":"1565","article-title":"Recurrent medulloepithelioma of the ciliary body: immunohistochemical characteristics","volume":"95","author":"Kivela\n              T,","year":"1988","unstructured":"Kivela\n              T, \n            \n              Tarkkanen\n              A\n            \n          . Recurrent medulloepithelioma of the ciliary body: immunohistochemical characteristics. 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Arch Ophthalmol. 1994;112(2):217\u2013221."},{"issue":"3","key":"arpa-136-07-829-831-b04","series-title":"Cancer","first-page":"725","article-title":"Extraskeletal Ewing's sarcoma","volume":"85","author":"Ahmad\n              R,","year":"1999","unstructured":"Ahmad\n              R, \n            \n              Mayol\n              BR, \n            \n              Davis\n              M\n            , et al. \n          Extraskeletal Ewing's sarcoma. Cancer. 1999;85(3):725\u2013731."},{"issue":"12","key":"arpa-136-07-829-831-b05","series-title":"Pediatr Radiol","first-page":"1365","article-title":"Extraosseous Ewing sarcoma of the thyroid gland","volume":"39","author":"Adapa\n              P,","year":"2009","unstructured":"Adapa\n              P, \n            \n              Chung\n              T, \n            \n              Popek\n              EJ\n            , et al. \n          Extraosseous Ewing sarcoma of the thyroid gland. 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Indian J Radiol Imaging. 2009;19(4):305\u2013307."},{"issue":"2","key":"arpa-136-07-829-831-b07","series-title":"Indian J Radiol Imaging","first-page":"151","article-title":"Extraosseous Ewing's sarcoma\/primitive neuroectodermal tumor of the sacral nerve plexus","volume":"19","author":"Narula\n              MK,","year":"2009","unstructured":"Narula\n              MK, \n            \n              Gupta\n              N, \n            \n              Anand\n              R\n            , et al. \n          Extraosseous Ewing's sarcoma\/primitive neuroectodermal tumor of the sacral nerve plexus. 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Am J Pathol. 1987;127(2):288\u2013304."},{"issue":"5","key":"arpa-136-07-829-831-b12","series-title":"Virchows Arch A Pathol Anat Histopathol","first-page":"421","article-title":"Malignant peripheral neuorectodermal tumors of bone other than Askin's neoplasm: characterization of 14 new cases with immunohistochemistry and electron microscopy","volume":"412","author":"Llombart-Bosch\n              A,","year":"1988","unstructured":"Llombart-Bosch\n              A, \n            \n              Lacombe\n              MJ, \n            \n              Peydro-Olaya\n              A\n            , et al. \n          Malignant peripheral neuorectodermal tumors of bone other than Askin's neoplasm: characterization of 14 new cases with immunohistochemistry and electron microscopy. Virchows Arch A Pathol Anat Histopathol. 1988;412(5):421\u2013430."},{"issue":"11","key":"arpa-136-07-829-831-b13","series-title":"Mod Pathol","first-page":"1175","article-title":"Differentiating lymphoblastic lymphoma from Ewing's sarcoma: lymphocyte markers and gene rearrangement","volume":"14","author":"Ozdemirli\n              M,","year":"2001","unstructured":"Ozdemirli\n              M, \n            \n              Fanburg-Smith\n              JC, \n            \n              Hartmann\n              DP\n            , et al. \n          Differentiating lymphoblastic lymphoma from Ewing's sarcoma: lymphocyte markers and gene rearrangement. Mod Pathol. 2001;14(11):1175\u20131182."},{"issue":"5","key":"arpa-136-07-829-831-b14","series-title":"N Engl J Med","first-page":"294","article-title":"The Ewing family of tumors\u2014a subgroup of small-round-cell tumors defined by specific chimeric transcripts","volume":"331","author":"Delattre\n              O,","year":"1994","unstructured":"Delattre\n              O, \n            \n              Zucman\n              J, \n            \n              Melot\n              T\n            , et al. \n          The Ewing family of tumors\u2014a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med. 1994;331(5):294\u2013299."},{"issue":"12","key":"arpa-136-07-829-831-b15","series-title":"Am J Surg Pathol","first-page":"1657","article-title":"Immunohistochemical detection of FLI-1 protein expression: a study of 132 round cell tumors with emphasis on CD99-positive mimics of Ewing's sarcoma\/primitive neuorectodermal tumor","volume":"24","author":"Folpe\n              AL,","year":"2000","unstructured":"Folpe\n              AL, \n            \n              Hill\n              CE, \n            \n              Parham\n              DM\n            , et al. \n          Immunohistochemical detection of FLI-1 protein expression: a study of 132 round cell tumors with emphasis on CD99-positive mimics of Ewing's sarcoma\/primitive neuorectodermal tumor. Am J Surg Pathol. 2000;24(12):1657\u20131662."},{"issue":"3","key":"arpa-136-07-829-831-b16","series-title":"Arch Ophthalmol","first-page":"207","article-title":"Metastasis of Ewing's sarcoma to the choroid","volume":"89","author":"Jampol\n              LM,","year":"1973","unstructured":"Jampol\n              LM, \n            \n              Cottle\n              E, \n            \n              Fischer\n              DS\n            , et al. \n          Metastasis of Ewing's sarcoma to the choroid. 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