Activated growth signaling pathway expression in Ewing sarcoma and clinical outcome

Pediatr Blood Cancer. 2012 Apr;58(4):532-8. doi: 10.1002/pbc.23348. Epub 2011 Oct 12.

Abstract

Background: In Ewing sarcoma (EWS) most of the research on signaling pathways has been performed on cell lines or animal models. The objective of the current study was to determine the relation between clinical outcome and the expression of proteins involved in active growth signaling pathways.

Methods: A paraffin-embedded microarray of 45 human primary EWS tissue specimens was stained with the antibodies against c-KIT, AKT, p-AKT, p-mTOR, IGF-1R, IGFBP-3, MAPK, p27(KIP1) , and p70S6 kinase. Immunohistochemical staining was correlated with patient overall survival (OS).

Results: In the univariate analysis 3 variables showed statistical significance to predict survival: presence of metastasis, p-mTOR, and p27(KIP1). A positive stain for p-mTOR (hazard ratio of 4.74 [95% CI (57, 121)]) was significantly (log-rank test with a P = 0.029) associated with better OS. Also, a positive stain for p27(KIP1) (hazard ratio of 6.87 [95% CI (77, 136)] was significantly (log-rank test with a P = 0.009) associated with better OS. Multivariate analysis showed metastasis (HR: 4.3; 95% CI: 0.99, 19; P = 0.05), p-mTOR (HR: 4.8 with 95% CI: 0.6, 38; P = 0.13) and p27 (HR: 5.3; 95% CI: 1.37, 20; P = 0.01) as independent prognostic factors of outcome.

Conclusions: In our series, p-mTOR and p27(KIP1) protein overexpression were independently associated with better survival.

Publication types

  • Clinical Trial
  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Disease-Free Survival
  • Female
  • Gene Expression Regulation, Neoplastic*
  • Humans
  • Infant
  • Insulin-Like Growth Factor Binding Protein 3 / biosynthesis
  • Intracellular Signaling Peptides and Proteins / metabolism
  • Male
  • Neoplasm Metastasis
  • Neoplasm Proteins / biosynthesis*
  • Protein Kinases / biosynthesis
  • Sarcoma, Ewing / drug therapy
  • Sarcoma, Ewing / metabolism*
  • Sarcoma, Ewing / mortality*
  • Sarcoma, Ewing / pathology
  • Signal Transduction*
  • Survival Rate

Substances

  • IGFBP3 protein, human
  • Insulin-Like Growth Factor Binding Protein 3
  • Intracellular Signaling Peptides and Proteins
  • Neoplasm Proteins
  • Protein Kinases