Thesis (Ph.D.) - Cairo University - National Cancer Institute - Department of Pediatric Oncology

Background: Survivin is an inhibitor of apoptosis protein that inhibits caspases and blocks cell death. It is undetectable in most normal adult tissues. High survivin expression has been detected in various tumors and has been correlated with therapy resistance and poor outcome. Objectives: We conducted this study to examine survivin expression in pediatric Ewing sarcoma family of tumors (ESFT), and evaluate its role in predicting clinical outcome. Patients and tnahods: Formalin-fixed paraffin-embedded tumor tissues from 108 pediatric patients with ESFT were examined for survivin status and percent of expression by immunostaining with survivin rabbit monoclonal antibodies. The relationship between survivin status and dinico-pathologic characteristics mod survival rates was evaluated. Results: Survival expression was detected in tumor tissues of 72 (66.7 %) patients. High expression (0.50%) was detected in only 18 (16.7%) patients.From all clinico-pathologic factors, positive survivin status was only strongly associated with male gender (p value= 0.041). In the Can proportional regression model analysis, high survivin expression was shown to be a significant univariate parameter for poorer overall (OS) and event free survival (EFS) with p value 0.033, and 0.037 respectively. Also, on multivariate analysis; it was an independent factor that significantly affect the OS (fi 0.041; BR 1.97 with 95% CI of 1.03-3.79), and EFS (p: 0.049; IIR 1.86 with 95% CI of 1.00-346). Conclusion: High expression of survivin in ESFT identifies a group of patients with poor prognosis.This result needs to be confirmed in prospective studies including larger number of patients thus survivin expression may in future help to refute risk adapted treatment

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