| Title: |
Atypical Neuroblastoma With Absent Urinary Catecholamine Excretion and 123 ImIBG Avidity Are of Favorable Outcome: A Retrospective French Single‐Center Study |
| Authors: |
Borovkov, Anna; Assy, Juliette; Aerts, Isabelle; Bourdeaut, Franck; Cordero, Camille; Laurence, Valérie; Leruste, Amaury; Winter, Sarah; Michon, Jean; Orbach, Daniel; Pacquement, Hélène; Philippe‐Chomette, Pascale; Pierron, Gaelle; Masliah‐Planchon, Julien; Helfre, Sylvie; Janoueix‐Lerosey, Isabelle; Defachelles, Anne‐Sophie; Pasqualini, Claudia; Jehanno, Nina; Cyrta, Joanna; Gauthier, Arnaud; Mosseri, Véronique; Doz, François; Sarnacki, Sabine; Cardoen, Liesbeth; Brisse, Hervé J.; Luporsi, Marie; Schleiermacher, Gudrun |
| Source: |
Pediatric Blood & Cancer ; volume 72, issue 11 ; ISSN 1545-5009 1545-5017 |
| Publisher Information: |
Wiley |
| Publication Year: |
2025 |
| Collection: |
Wiley Online Library (Open Access Articles via Crossref) |
| Description: |
Background In neuroblastoma (NB), urinary catecholamine excretion and 123 ImIBG 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 123 ImIBG avidity, remains to be determined. We sought to determine the frequency and prognosis of atypical NB and investigate the significance of catecholamine profiles and 123 ImIBG avidity at diagnosis. Methods From 2000 to 2020, 275 children with NB, aged 0–20 years at diagnosis, treated at Institut Curie, France, were retrospectively analyzed. Results Overall, 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. Conclusions Atypical 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. |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| DOI: |
10.1002/pbc.32025 |
| Availability: |
https://doi.org/10.1002/pbc.32025; https://onlinelibrary.wiley.com/doi/pdf/10.1002/pbc.32025 |
| Rights: |
http://creativecommons.org/licenses/by/4.0/ |
| Accession Number: |
edsbas.A5D97C16 |
| Database: |
BASE |