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Primary Ewing sarcoma/peripheral primitive neuroectodermal tumor of the vulva.
Hum Pathol. 2008 Oct; 39(10):1535-9.HP

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.

Authors+Show Affiliations

Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA.No affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

18602673

Citation

Fong, Y Erika, et al. "Primary Ewing Sarcoma/peripheral Primitive Neuroectodermal Tumor of the Vulva." Human Pathology, vol. 39, no. 10, 2008, pp. 1535-9.
Fong YE, López-Terrada D, Zhai QJ. Primary Ewing sarcoma/peripheral primitive neuroectodermal tumor of the vulva. Hum Pathol. 2008;39(10):1535-9.
Fong, Y. E., López-Terrada, D., & Zhai, Q. J. (2008). Primary Ewing sarcoma/peripheral primitive neuroectodermal tumor of the vulva. Human Pathology, 39(10), 1535-9. https://doi.org/10.1016/j.humpath.2008.01.013
Fong YE, López-Terrada D, Zhai QJ. Primary Ewing Sarcoma/peripheral Primitive Neuroectodermal Tumor of the Vulva. Hum Pathol. 2008;39(10):1535-9. PubMed PMID: 18602673.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Primary Ewing sarcoma/peripheral primitive neuroectodermal tumor of the vulva. AU - Fong,Y Erika, AU - López-Terrada,Dolores, AU - Zhai,Qihui Jim, Y1 - 2008/07/07/ PY - 2007/10/11/received PY - 2008/01/16/revised PY - 2008/01/17/accepted PY - 2008/7/8/pubmed PY - 2008/9/24/medline PY - 2008/7/8/entrez SP - 1535 EP - 9 JF - Human pathology JO - Hum Pathol VL - 39 IS - 10 N2 - 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. SN - 1532-8392 UR - https://www.unboundmedicine.com/medline/citation/18602673/Primary_Ewing_sarcoma/peripheral_primitive_neuroectodermal_tumor_of_the_vulva_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0046-8177(08)00059-2 DB - PRIME DP - Unbound Medicine ER -