Survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST).

Survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST).

Malignant peripheral nerve sheath tumors (MPNST) are very aggressive malignancies comprising approximately 5-10% of all soft tissue sarcomas. In this study, we focused on pediatric MPNST arising in the first 2 decades of life, as they represent one the most frequent non-rhabdomyosarcomatous soft tis...

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Autores principales: Rita Alaggio, Riccardo Turrini, Daniela Boldrin, Anna Merlo, Claudio Gambini, Andrea Ferrari, Patrizia Dall'igna, Cheryl M Coffin, Annalisa Martines, Laura Bonaldi, Gian Luca De Salvo, Paola Zanovello, Antonio Rosato
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Lenguaje:EN
Publicado: Public Library of Science (PLoS) 2013
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Acceso en línea:https://doaj.org/article/3b2610436ef346b2aaaba46f9c92e570
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spelling oai:doaj.org-article:3b2610436ef346b2aaaba46f9c92e5702021-11-18T08:44:37ZSurvivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST).1932-620310.1371/journal.pone.0080456https://doaj.org/article/3b2610436ef346b2aaaba46f9c92e5702013-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/24303016/pdf/?tool=EBIhttps://doaj.org/toc/1932-6203Malignant peripheral nerve sheath tumors (MPNST) are very aggressive malignancies comprising approximately 5-10% of all soft tissue sarcomas. In this study, we focused on pediatric MPNST arising in the first 2 decades of life, as they represent one the most frequent non-rhabdomyosarcomatous soft tissue sarcomas in children. In MPNST, several genetic alterations affect the chromosomal region 17q encompassing the BIRC5/SURVIVIN gene. As cancer-specific expression of survivin has been found to be an effective marker for cancer detection and outcome prediction, we analyzed survivin expression in 35 tumor samples derived from young patients affected by sporadic and neurofibromatosis type 1-associated MPNST. Survivin mRNA and protein expression were assessed by Real-Time PCR and immunohistochemical staining, respectively, while gene amplification was analyzed by FISH. Data were correlated with the clinicopathological characteristics of patients. Survivin mRNA was overexpressed in pediatric MPNST and associated to a copy number gain of BIRC5; furthermore, increased levels of transcripts correlated with a higher FNCLCC tumor grade (grade 1 and 2 vs. 3, p = 0.0067), and with a lower survival probability (Log-rank test, p = 0.0038). Overall, these data support the concept that survivin can be regarded as a useful prognostic marker for pediatric MPNST and a promising target for therapeutic interventions.Rita AlaggioRiccardo TurriniDaniela BoldrinAnna MerloClaudio GambiniAndrea FerrariPatrizia Dall'ignaCheryl M CoffinAnnalisa MartinesLaura BonaldiGian Luca De SalvoPaola ZanovelloAntonio RosatoPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 8, Iss 11, p e80456 (2013)
institution DOAJ
collection DOAJ
language EN
topic Medicine
R
Science
Q
spellingShingle Medicine
R
Science
Q
Rita Alaggio
Riccardo Turrini
Daniela Boldrin
Anna Merlo
Claudio Gambini
Andrea Ferrari
Patrizia Dall'igna
Cheryl M Coffin
Annalisa Martines
Laura Bonaldi
Gian Luca De Salvo
Paola Zanovello
Antonio Rosato
Survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST).
description Malignant peripheral nerve sheath tumors (MPNST) are very aggressive malignancies comprising approximately 5-10% of all soft tissue sarcomas. In this study, we focused on pediatric MPNST arising in the first 2 decades of life, as they represent one the most frequent non-rhabdomyosarcomatous soft tissue sarcomas in children. In MPNST, several genetic alterations affect the chromosomal region 17q encompassing the BIRC5/SURVIVIN gene. As cancer-specific expression of survivin has been found to be an effective marker for cancer detection and outcome prediction, we analyzed survivin expression in 35 tumor samples derived from young patients affected by sporadic and neurofibromatosis type 1-associated MPNST. Survivin mRNA and protein expression were assessed by Real-Time PCR and immunohistochemical staining, respectively, while gene amplification was analyzed by FISH. Data were correlated with the clinicopathological characteristics of patients. Survivin mRNA was overexpressed in pediatric MPNST and associated to a copy number gain of BIRC5; furthermore, increased levels of transcripts correlated with a higher FNCLCC tumor grade (grade 1 and 2 vs. 3, p = 0.0067), and with a lower survival probability (Log-rank test, p = 0.0038). Overall, these data support the concept that survivin can be regarded as a useful prognostic marker for pediatric MPNST and a promising target for therapeutic interventions.
format article
author Rita Alaggio
Riccardo Turrini
Daniela Boldrin
Anna Merlo
Claudio Gambini
Andrea Ferrari
Patrizia Dall'igna
Cheryl M Coffin
Annalisa Martines
Laura Bonaldi
Gian Luca De Salvo
Paola Zanovello
Antonio Rosato
author_facet Rita Alaggio
Riccardo Turrini
Daniela Boldrin
Anna Merlo
Claudio Gambini
Andrea Ferrari
Patrizia Dall'igna
Cheryl M Coffin
Annalisa Martines
Laura Bonaldi
Gian Luca De Salvo
Paola Zanovello
Antonio Rosato
author_sort Rita Alaggio
title Survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST).
title_short Survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST).
title_full Survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST).
title_fullStr Survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST).
title_full_unstemmed Survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST).
title_sort survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (mpnst).
publisher Public Library of Science (PLoS)
publishDate 2013
url https://doaj.org/article/3b2610436ef346b2aaaba46f9c92e570
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