Abstract
BackgroundEnhancer 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.MethodsWe 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.ResultsEnhancer 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.ConclusionsOur 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.
Highlights
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
EZH2 overexpression was firstly linked to cancer by microarray analysis of prostate and breast cancer where it is associated to Ramaglia et al Cancer Cell Int (2016) 16:59 aggressive, metastatic disease and to a poor clinical outcome [3,4,5,6,7]
We have studied the expression of the polycomb complex proteins EZH2, SUZ12 and Ectoderm Development (EED) in paediatric soft tissue sarcomas correlating the expression with the clinical outcome of the patients
Summary
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. 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. The polycomb proteins retain the expression pattern of different cells by controlling chromatin structure during early development. PRC2 complex, conserved from Drosophila to mammals, consists of Enhancer of Zeste. Drosophila Homologue 2 (EZH2), Suppressor of Zeste (SUZ12) and Embryonic Ectoderm Development (EED). Polycomb repressive complex 2 catalyses the methylation of lysine 27 at histone H3, a repressive mark, and is involved in many biological processes as differentiation, cell identity, proliferation and stem-cell plasticity [1]. Overexpression of EZH2 has been described in other adult cancers including bladder, gastric, lung, and hepatocellular carcinoma [8]
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