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| Home > Publications database > Tackling ALT-positive neuroblastoma: is it time to redefine risk classification systems? A systematic review with IPD meta-analysis. |
| Home > Publications database > Tackling ALT-positive neuroblastoma: is it time to redefine risk classification systems? A systematic review with IPD meta-analysis. |
| Journal Article (Review Article) | DKFZ-2025-00023 |
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2025
Stockton Press
Basingstoke
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Please use a persistent id in citations: doi:10.1016/j.neo.2024.101106
Abstract: The heterogeneous prognosis in neuroblastoma, shaped by telomere maintenance mechanisms (TMMs), notably the alternative lengthening of telomeres (ALT) pathway, necessitates a refined risk classification for high-risk patients. Current systems often lack precision, hindering tailored treatment approaches. This individual participant data (IPD) meta-analysis of survival among ALT-positive patients aims to improve risk classification systems, enhancing therapeutic strategies and patient outcomes.Following PRISMA-IPD guidelines, we conducted a comprehensive review of neuroblastoma patients retrieved from PubMed, Scopus, and Embase databases until March-2024. Patients were stratified into ALT-positive and TMM-negative subgroups. Overall and event-free survival probabilities were evaluated.In our cohort of 293 patients (156 ALT-positive, 137 TMM-negative) obtained from eight different studies, ALT-positive individuals displayed lower survival rates than TMM-negative patients. Non-stage 4 ALT-positive patients had reduced overall and event-free survival probabilities compared to their TMM-negative counterparts, indicating potential misclassification. Stage 4 ALT-positive patients similarly showed poorer survival outcomes than non-stage 4 TMM-negative patients, underscoring the significance of ALT in patient prognosis.Our study highlights poorer outcomes in ALT-positive neuroblastoma patients, emphasizing the need to integrate TMM status into international risk classification guidelines. Standardizing TMM assessment is key for refining treatment strategies, considering the unique biology of ALT-positive patients.
Keyword(s): Childhood cancer ; Prognosis biomarker ; Survival ; Telomere maintenance mechanisms ; Therapeutic strategies
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