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Primitive neuroectodermal tumor originating in the vulva: A case report

Primitive neuroectodermal tumors (PNETs) exhibit chromosomal translocations in common with those of Ewing’s sarcoma. They usually originate in bone or soft tissue but rarely arise in the vulva. The current case report presents a case of PNET originating in the vulva in a 60-year-old female, who prev...

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Autores principales: MATSUDA, MAKIKO, ICHIMURA, TOMOYUKI, KASAI, MARI, MURAKAMI, MAKOTO, HOSHI, MANABU, KAWAMURA, NAOKI, SUMI, TOSHIYUKI
Formato: Online Artículo Texto
Lenguaje:English
Publicado: D.A. Spandidos 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4063582/
https://www.ncbi.nlm.nih.gov/pubmed/24959242
http://dx.doi.org/10.3892/ol.2014.2073
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author MATSUDA, MAKIKO
ICHIMURA, TOMOYUKI
KASAI, MARI
MURAKAMI, MAKOTO
HOSHI, MANABU
KAWAMURA, NAOKI
SUMI, TOSHIYUKI
author_facet MATSUDA, MAKIKO
ICHIMURA, TOMOYUKI
KASAI, MARI
MURAKAMI, MAKOTO
HOSHI, MANABU
KAWAMURA, NAOKI
SUMI, TOSHIYUKI
author_sort MATSUDA, MAKIKO
collection PubMed
description Primitive neuroectodermal tumors (PNETs) exhibit chromosomal translocations in common with those of Ewing’s sarcoma. They usually originate in bone or soft tissue but rarely arise in the vulva. The current case report presents a case of PNET originating in the vulva in a 60-year-old female, who previously underwent enucleation of a vulvar tumor in another hospital. The pathologist suspected a histopathological diagnosis of PNET, and simple vulvectomy and resection of the inguinal lymph nodes were performed. An ~3 cm mass recurred in the right side of the vulva four years following the initial surgery and the tumor was excised. The tumor comprised small, round-to-oval nuclei and stained positively for MIC-2, synaptophysin, neuron-specific enolase and neurofilament antibodies. To date, the patient remains alive and with no evidence of disease four years following multidisciplinary treatment, despite PNETs usually exhibiting a poor prognosis. This is due to the small tumor size and the absence of distant metastasis.
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spelling pubmed-40635822014-06-23 Primitive neuroectodermal tumor originating in the vulva: A case report MATSUDA, MAKIKO ICHIMURA, TOMOYUKI KASAI, MARI MURAKAMI, MAKOTO HOSHI, MANABU KAWAMURA, NAOKI SUMI, TOSHIYUKI Oncol Lett Articles Primitive neuroectodermal tumors (PNETs) exhibit chromosomal translocations in common with those of Ewing’s sarcoma. They usually originate in bone or soft tissue but rarely arise in the vulva. The current case report presents a case of PNET originating in the vulva in a 60-year-old female, who previously underwent enucleation of a vulvar tumor in another hospital. The pathologist suspected a histopathological diagnosis of PNET, and simple vulvectomy and resection of the inguinal lymph nodes were performed. An ~3 cm mass recurred in the right side of the vulva four years following the initial surgery and the tumor was excised. The tumor comprised small, round-to-oval nuclei and stained positively for MIC-2, synaptophysin, neuron-specific enolase and neurofilament antibodies. To date, the patient remains alive and with no evidence of disease four years following multidisciplinary treatment, despite PNETs usually exhibiting a poor prognosis. This is due to the small tumor size and the absence of distant metastasis. D.A. Spandidos 2014-07 2014-04-16 /pmc/articles/PMC4063582/ /pubmed/24959242 http://dx.doi.org/10.3892/ol.2014.2073 Text en Copyright © 2014, Spandidos Publications http://creativecommons.org/licenses/by/3.0 This is an open-access article licensed under a Creative Commons Attribution-NonCommercial 3.0 Unported License. The article may be redistributed, reproduced, and reused for non-commercial purposes, provided the original source is properly cited.
spellingShingle Articles
MATSUDA, MAKIKO
ICHIMURA, TOMOYUKI
KASAI, MARI
MURAKAMI, MAKOTO
HOSHI, MANABU
KAWAMURA, NAOKI
SUMI, TOSHIYUKI
Primitive neuroectodermal tumor originating in the vulva: A case report
title Primitive neuroectodermal tumor originating in the vulva: A case report
title_full Primitive neuroectodermal tumor originating in the vulva: A case report
title_fullStr Primitive neuroectodermal tumor originating in the vulva: A case report
title_full_unstemmed Primitive neuroectodermal tumor originating in the vulva: A case report
title_short Primitive neuroectodermal tumor originating in the vulva: A case report
title_sort primitive neuroectodermal tumor originating in the vulva: a case report
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4063582/
https://www.ncbi.nlm.nih.gov/pubmed/24959242
http://dx.doi.org/10.3892/ol.2014.2073
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