Browse by author
Lookup NU author(s): Dr Nicholas Bown, Professor John LunecORCiD, Professor Deborah Tweddle
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND).
PURPOSE: For localized, resectable neuroblastoma without MYCN amplification, surgery only is recommended even if incomplete. However, it is not known whether the genomic background of these tumors may influence outcome. PATIENTS AND METHODS: Diagnostic samples were obtained from 317 tumors, International Neuroblastoma Staging System stages 1/2A/2B, from 3 cohorts: Localized Neuroblastoma European Study Group I/II and Children's Oncology Group. Genomic data were analyzed using multi- and pangenomic techniques and fluorescence in-situ hybridization in 2 age groups (cutoff age, 18 months) and were quality controlled by the International Society of Pediatric Oncology European Neuroblastoma (SIOPEN) Biology Group. RESULTS: Patients with stage 1 tumors had an excellent outcome (5-year event-free survival [EFS] ± standard deviation [SD], 95% ± 2%; 5-year overall survival [OS], 99% ± 1%). In contrast, patients with stage 2 tumors had a reduced EFS in both age groups (5-year EFS ± SD, 84% ± 3% in patients < 18 months of age and 75% ± 7% in patients ≥ 18 months of age). However, OS was significantly decreased only in the latter group (5-year OS ± SD in < 18months and ≥ 18months, 96% ± 2% and 81% ± 7%, respectively; P = .001). In < 18months, relapses occurred independent of segmental chromosome aberrations (SCAs); only 1p loss decreased EFS (5-year EFS ± SD in patients 1p loss and no 1p loss, 62% ± 13% and 87% ± 3%, respectively; P = .019) but not OS (5-year OS ± SD, 92% ± 8% and 97% ± 2%, respectively). In patients ≥ 18 months, only SCAs led to relapse and death, with 11q loss as the strongest marker (11q loss and no 11q loss: 5-year EFS ± SD, 48% ± 16% and 85% ± 7%, P = .033; 5-year OS ± SD, 46% ± 22% and 92% ± 6%, P = .038). CONCLUSION: Genomic aberrations of resectable non-MYCN-amplified stage 2 neuroblastomas have a distinct age-dependent prognostic impact. Chromosome 1p loss is a risk factor for relapse but not for diminished OS in patients < 18 months, SCAs (especially 11q loss) are risk factors for reduced EFS and OS in those > 18months. In older patients with SCA, a randomized trial of postoperative chemotherapy compared with observation alone may be indicated.
Author(s): Ambros IM, Tonini G-P, Potschger U, Gross N, Mosseri V, Beiske K, Berbegall AP, Benard J, Bown N, Caron H, Combaret V, Couturier J, Defferrari R, Delattre O, Jeison M, Kogner P, Lunec J, Marques B, Martinsson T, Mazzocco K, Noguera R, Schleiermacher G, Valent A, Van Roy N, Villamon E, Janousek D, Pribill I, Glogova E, Attiyeh EF, Hogarty MD, Monclair TF, Holmes K, Valteau-Couanet D, Castel V, Tweddle DA, Park JR, Cohn S, Ladenstein R, Beck-Popovic M, De Bernardi B, Michon J, Pearson ADJ, Ambros PF
Publication type: Article
Publication status: Published
Journal: Journal of Clinical Oncology
Year: 2020
Volume: 38
Issue: 31
Pages: 3685-3697
Print publication date: 01/11/2020
Online publication date: 09/09/2020
Acceptance date: 07/08/2020
Date deposited: 13/01/2021
ISSN (print): 0732-183X
ISSN (electronic): 1527-7755
Publisher: American Society of Clinical Oncology
URL: https://doi.org/10.1200/JCO.18.02132
DOI: 10.1200/JCO.18.02132
PubMed id: 32903140
Altmetrics provided by Altmetric
