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000296075 1001_ $$0P:(DE-HGF)0$$aAvinent-Pérez, Marta$$b0$$eFirst author
000296075 245__ $$aTackling ALT-positive neuroblastoma: is it time to redefine risk classification systems? A systematic review with IPD meta-analysis.
000296075 260__ $$aBasingstoke$$bStockton Press$$c2025
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000296075 520__ $$aThe heterogeneous prognosis in neuroblastoma, shaped by telomere maintenance mechanisms (TMMs), notably the alternative lengthening of telomeres (ALT) pathway, necessitates a refined risk classification for high-risk patients. Current systems often lack precision, hindering tailored treatment approaches. This individual participant data (IPD) meta-analysis of survival among ALT-positive patients aims to improve risk classification systems, enhancing therapeutic strategies and patient outcomes.Following PRISMA-IPD guidelines, we conducted a comprehensive review of neuroblastoma patients retrieved from PubMed, Scopus, and Embase databases until March-2024. Patients were stratified into ALT-positive and TMM-negative subgroups. Overall and event-free survival probabilities were evaluated.In our cohort of 293 patients (156 ALT-positive, 137 TMM-negative) obtained from eight different studies, ALT-positive individuals displayed lower survival rates than TMM-negative patients. Non-stage 4 ALT-positive patients had reduced overall and event-free survival probabilities compared to their TMM-negative counterparts, indicating potential misclassification. Stage 4 ALT-positive patients similarly showed poorer survival outcomes than non-stage 4 TMM-negative patients, underscoring the significance of ALT in patient prognosis.Our study highlights poorer outcomes in ALT-positive neuroblastoma patients, emphasizing the need to integrate TMM status into international risk classification guidelines. Standardizing TMM assessment is key for refining treatment strategies, considering the unique biology of ALT-positive patients.
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000296075 650_7 $$2Other$$aChildhood cancer
000296075 650_7 $$2Other$$aPrognosis biomarker
000296075 650_7 $$2Other$$aSurvival
000296075 650_7 $$2Other$$aTelomere maintenance mechanisms
000296075 650_7 $$2Other$$aTherapeutic strategies
000296075 7001_ $$0P:(DE-He78)91f32735ee876c579d63c05a7f4778dd$$aWestermann, Frank$$b1$$udkfz
000296075 7001_ $$aNavarro, Samuel$$b2
000296075 7001_ $$aLópez-Carrasco, Amparo$$b3
000296075 7001_ $$aNoguera, Rosa$$b4
000296075 773__ $$0PERI:(DE-600)2008231-9$$a10.1016/j.neo.2024.101106$$gVol. 60, p. 101106 -$$p101106$$tNeoplasia$$v60$$x1522-8002$$y2025
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