Objective Neuroblastoma is the most common, deadly extracranial solid tumour in childhood. It sometime shows spontaneous regression and/or matures into a benign ganglioneuroma. Most patients older than one year have extensive disease at presentation, with poor overall survival. This heterogeneity defied explanation until the recent advances in the field of molecular genetics. We assessed the prognostic value of telomerase activity and N-MYC gene amplification in a set of neuroblastoma patients.
Methods Forty-four patients were studied at the Paediatric Department, National Cancer Institute, Egypt. Patients were treated according to risk stratified protocols and followed up for at least 2 years. Telomerase activity was estimated by immunohistochemistry, N-MYC amplification was assessed by chromogen in situ hybridisation and reverse transcriptase–PCR and was correlated with disease response, survival and standard clinicopathological prognostic factors.
Results Twenty-three cases (52.27%) had high telomerase expression and 21 (47.27%) had low expression. None of the cases with high telomerase activity entered into complete remission compared with 13 (61.9%) patients with low telomerase activity (p<0.0001). N-MYC gene amplification was detected in 39 (88.6%) cases. Significant correlations were reported between high telomerase expression, N-MYC gene amplification and poor prognostic factors. Reduced overall survival was significantly correlated with reduced overall survival (p<0.0001).
Conclusions Telomerase activity is a potential prognostic factor in paediatric neuroblastoma that should be included as a part of the initial evaluation of patients. However, further extended studies with longer follow-up periods are required.
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