Abstract
We report the clinical, histologic, immunohistochemical, and molecular findings of a Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) localized to the rectum in a 17-year-old boy. Notably the 4.5 x 4 x 4-cm sessile mass was spontaneously eliminated through the anus, producing an episode of hemorrhagic shock. Histologically the tumor presented as a proliferation of membrane CD99-positive, small round cells. EWS/FLI1 chimeric mRNA was demonstrated by nested reverse-transcription polymerase chain reaction (RT-PCR) analysis in the tumor tissue. The patient is alive and well 2 years after initial symptoms. We also review reported cases of ES/PNET of the digestive system.
MeSH terms
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12E7 Antigen
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Adolescent
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Antigens, CD / metabolism
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Biomarkers, Tumor
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Cell Adhesion Molecules / metabolism
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DNA-Binding Proteins / metabolism
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Humans
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Male
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Neuroectodermal Tumors, Primitive, Peripheral / diagnosis
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Neuroectodermal Tumors, Primitive, Peripheral / genetics
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Neuroectodermal Tumors, Primitive, Peripheral / metabolism
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Neuroectodermal Tumors, Primitive, Peripheral / pathology*
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Proto-Oncogene Protein c-fli-1
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Rectal Neoplasms / diagnosis
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Rectal Neoplasms / genetics
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Rectal Neoplasms / metabolism
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Rectal Neoplasms / pathology*
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Sarcoma, Ewing / diagnosis
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Sarcoma, Ewing / genetics
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Sarcoma, Ewing / metabolism
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Sarcoma, Ewing / pathology*
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Trans-Activators / metabolism
Substances
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12E7 Antigen
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Antigens, CD
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Biomarkers, Tumor
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CD99 protein, human
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Cell Adhesion Molecules
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DNA-Binding Proteins
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FLI1 protein, human
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Proto-Oncogene Protein c-fli-1
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Trans-Activators