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Published in: Cancer Cell International 1/2016

Open Access 01-12-2016 | Primary research

High EZH2 expression is correlated to metastatic disease in pediatric soft tissue sarcomas

Authors: Maria Ramaglia, Velia D’Angelo, Adriana Iannotta, Daniela Di Pinto, Elvira Pota, Maria Carmen Affinita, Vittoria Donofrio, Maria Elena Errico, Angela Lombardi, Cristiana Indolfi, Fiorina Casale, Michele Caraglia

Published in: Cancer Cell International | Issue 1/2016

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Abstract

Background

Enhancer of Zeste Drosophila Homologue 2 (EZH2) is a key regulator of transcription as a member of polycomb repressive complex 2 (PRC2) which exerts repression of downstream genes and is correlated to invasiveness and progression of different tumours. Therefore, we evaluated the expression of PRC2 proteins in pediatric soft tissue sarcoma (rhabdomyosarcoma, RMS and extraosseous Ewing sarcoma, EES) correlating them to the clinical outcome of the patients.

Methods

We analyzed PRC2 protein expression by quantitative real time PCR, western blotting and immunohistochemistry in 17 soft tissue sarcomas (11 RMS and 6 EES) enrolled at Paediatric Oncology Units of the Second University of Naples. Expression analysis was performed for EZH2, SUZ12 and EED.

Results

Enhancer of Zeste Drosophila Homologue 2 was expressed with a different degree in 60 % of samples. Interestingly, the magnitude of EZH2 up regulation was significantly higher in patients presenting lymph node and/or distant metastases at the diagnosis. Moreover, patients overexpressing EZH2 had a lower probability of survival compared to patients negative or with low EZH2 expression.

Conclusions

Our study suggests that high EZH2 expression is associated to increased aggressiveness of the disease. Therefore, drugs that control its activity could be potentially used in the epigenetic target treatment of tumors with these alterations.
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Metadata
Title
High EZH2 expression is correlated to metastatic disease in pediatric soft tissue sarcomas
Authors
Maria Ramaglia
Velia D’Angelo
Adriana Iannotta
Daniela Di Pinto
Elvira Pota
Maria Carmen Affinita
Vittoria Donofrio
Maria Elena Errico
Angela Lombardi
Cristiana Indolfi
Fiorina Casale
Michele Caraglia
Publication date
01-12-2016
Publisher
BioMed Central
Published in
Cancer Cell International / Issue 1/2016
Electronic ISSN: 1475-2867
DOI
https://doi.org/10.1186/s12935-016-0338-x

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