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| Main Authors: | , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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| Format: | Artículo Open Access |
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Wiley
2025
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| Online Access: | https://onlinelibrary.wiley.com/doi/10.1002/pbc.32025 |
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Table of Contents:
- Atypical Neuroblastoma With Absent Urinary Catecholamine Excretion and 123ImIBG Avidity Are of Favorable Outcome: A Retrospective French Single‐Center Study Anna Borovkov Juliette Assy Isabelle Aerts Franck Bourdeaut Camille Cordero Valérie Laurence Amaury Leruste Sarah Winter Jean Michon Daniel Orbach Hélène Pacquement Pascale Philippe‐Chomette Gaelle Pierron Julien Masliah‐Planchon Sylvie Helfre Isabelle Janoueix‐Lerosey Anne‐Sophie Defachelles Claudia Pasqualini Nina Jehanno Joanna Cyrta Arnaud Gauthier Véronique Mosseri François Doz Sabine Sarnacki Liesbeth Cardoen Hervé J. Brisse Marie Luporsi Gudrun Schleiermacher Pediatric Blood & Cancer AbstractBackgroundIn neuroblastoma (NB), urinary catecholamine excretion and 123ImIBG avidity—depending on tumor enzymatic activity and norepinephrine transporter expression, respectively—are diagnostic standards. The prognostic impact of atypical NB, without urinary catecholamine excretion and/or 123ImIBG avidity, remains to be determined. We sought to determine the frequency and prognosis of atypical NB and investigate the significance of catecholamine profiles and 123ImIBG avidity at diagnosis.MethodsFrom 2000 to 2020, 275 children with NB, aged 0–20 years at diagnosis, treated at Institut Curie, France, were retrospectively analyzed.ResultsOverall, 24% of NB had atypical features (n = 67/275). Lower INRG stages L1/L2 were more frequent in atypical NB, 66% versus 28% (n = 44/67 vs. 59/208), with less INRG Stage M than in typical NB, 25% versus 61% (n = 17/67 vs. 126/208), p < 0.001. Atypical tumors more frequently harbored favorable molecular features with less frequent MYCN amplification, 12% (n = 8/64) versus 29% (n = 58/201), p < 0.01, and fewer cases with segmental chromosomal alterations, 30% (n = 13/44) versus 60% (n = 69/115), p < 0.05. Event‐free survival (EFS) and overall survival (OS) were better in atypical than typical NB (5‐year EFS: 77% ± 5% vs. 50% ± 4% and OS 87% ± 4% vs. 65% ± 4%, p < 0.001). However, in multivariate analysis, atypical features in NB were not significant independent markers of prognosis.ConclusionsAtypical NB constitute a subgroup of interest for biomolecular analyses, including transcriptomics, which might provide further insights into disease‐associated molecular features and our understanding of NB development. 10.1002/pbc.32025 http://creativecommons.org/licenses/by/4.0/