Abstract
We report a case of Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) arising in the vulva in a 17-year-old adolescent girl. Primary extraskeletal ES/pPNET is uncommon and only rarely affects the female genital tract. Characteristic histologic features of ES/pPNET were present in this case including a monomorphic population of small round blue cells with cytoplasmic glycogen confirmed by periodic acid-Schiff and were immunohistochemically positive for CD99 in a membranous and Fli-1 in a nuclear pattern. The presence of an EWS/Fli-1 fusion transcript was demonstrated by reverse transcription polymerase chain reaction and confirmed by direct sequencing. This report described another very rare case of ES/pPNET arising in the vulva and confirmed by molecular analysis.
MeSH terms
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Adolescent
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Base Sequence
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Biomarkers, Tumor
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DNA, Neoplasm / analysis
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Female
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Glycogen / metabolism
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Humans
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Molecular Sequence Data
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Neoplasms, Multiple Primary
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Neuroectodermal Tumors, Primitive, Peripheral / metabolism
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Neuroectodermal Tumors, Primitive, Peripheral / pathology*
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Oncogene Proteins, Fusion / genetics
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Oncogene Proteins, Fusion / metabolism
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Periodic Acid-Schiff Reaction
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Proto-Oncogene Protein c-fli-1
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RNA-Binding Protein EWS
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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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Sequence Analysis, DNA
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Transcription Factors / genetics
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Transcription Factors / metabolism
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Vulvar Neoplasms / genetics
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Vulvar Neoplasms / metabolism
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Vulvar Neoplasms / pathology*
Substances
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Biomarkers, Tumor
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DNA, Neoplasm
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EWS-FLI fusion protein
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Oncogene Proteins, Fusion
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Proto-Oncogene Protein c-fli-1
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RNA-Binding Protein EWS
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Transcription Factors
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Glycogen