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

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

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Article numbere80456
JournalPLoS One
Volume8
Issue number11
DOIs
Publication statusPublished - Nov 26 2013

ASJC Scopus subject areas

  • Agricultural and Biological Sciences(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • Medicine(all)

Fingerprint

Dive into the research topics of 'Survivin expression and prognostic significance in pediatric malignant peripheral nerve sheath tumors (MPNST)'. Together they form a unique fingerprint.

Cite this