TY  - JOUR
AU  - Avinent-Pérez, Marta
AU  - Westermann, Frank
AU  - Navarro, Samuel
AU  - López-Carrasco, Amparo
AU  - Noguera, Rosa
TI  - Tackling ALT-positive neuroblastoma: is it time to redefine risk classification systems? A systematic review with IPD meta-analysis.
JO  - Neoplasia
VL  - 60
SN  - 1522-8002
CY  - Basingstoke
PB  - Stockton Press
M1  - DKFZ-2025-00023
SP  - 101106
PY  - 2025
N1  - #EA:B087#
AB  - 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.
KW  - Childhood cancer (Other)
KW  - Prognosis biomarker (Other)
KW  - Survival (Other)
KW  - Telomere maintenance mechanisms (Other)
KW  - Therapeutic strategies (Other)
LB  - PUB:(DE-HGF)16
C6  - pmid:39733691
DO  - DOI:10.1016/j.neo.2024.101106
UR  - https://inrepo02.dkfz.de/record/296075
ER  -