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Management and Outcome of Stage 3 Neuroblastoma

Overview
Journal Eur J Cancer
Specialty Oncology
Date 2008 Nov 11
PMID 18996003
Citations 15
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Abstract

Purpose: The management of patients with International Neuroblastoma Staging System (INSS) stage 3 neuroblastoma (NB) is not consistent worldwide. We describe a single centre approach at Memorial Sloan-Kettering Cancer Centre (MSKCC) from 1991 to 2007 that minimises therapy except for those patients with MYCN-amplified NB.

Methods: In this retrospective analysis of 69 patients, tumour MYCN was not amplified in 53 and amplified in 16. Event-free survival (EFS) and overall survival (OS) were determined by Kaplan-Meier analysis.

Results: Fourteen patients with non-MYCN-amplified tumours were treated with surgery alone (group A) and the remaining 39 (group B) with surgery following chemotherapy that was initiated and administered at non-MSKCC institutions. Chemotherapy was discontinued after surgery in 38/39 of the latter. The 10-year EFS and OS for all patients with MYCN-non-amplified NB were 74.9+/-16.9% and 92.6+/-5.5%, respectively. There was no difference in OS between groups A and B (p=0.2; 10-year OS for groups A and B was 84.6+/-14% and 97.1+/-2.9%, respectively). Patients with MYCN-amplified disease (group C) underwent dose-intensive induction, tumour resection and local radiotherapy: 13 achieved complete or very good partial remission, and 10 received myeloablative chemotherapy. 11/16 patients also received 3F8-based immunotherapy: 10 remain free of disease. The 10-year EFS and OS for patients with MYCN-amplified neuroblastoma treated with immunotherapy were both 90.9+/-8.7%.

Conclusion: Patients with MYCN-non-amplified stage 3 NB can be successfully treated with surgery without the need for radiotherapy or continuation of chemotherapy. Combination of dose-intensive chemotherapy, surgery, radiotherapy and immunotherapy was associated with a favourable outcome for most patients with MYCN-amplified stage 3 NB.

Citing Articles

Survival of Patients With Neuroblastoma After Assignment to Reduced Therapy Because of the 12- to 18-Month Change in Age Cutoff in Children's Oncology Group Risk Stratification.

Bender H, Irwin M, Hogarty M, Castleberry R, Maris J, Kao P J Clin Oncol. 2023; 41(17):3149-3159.

PMID: 37098238 PMC: 10256433. DOI: 10.1200/JCO.22.01946.


Segmental chromosomal aberrations as the poor prognostic factor in children over 18 months with stage 3 neuroblastoma without amplification.

Wieczorek A, Szewczyk K, Klekawka T, Stefanowicz J, Ussowicz M, Drabik G Front Oncol. 2023; 13:1134772.

PMID: 36865795 PMC: 9972431. DOI: 10.3389/fonc.2023.1134772.


Impact of Genomic and Clinical Factors on Outcome of Children ≥18 Months of Age with Stage 3 Neuroblastoma with Unfavorable Histology and without MYCN Amplification: A Children's Oncology Group (COG) Report.

Pinto N, Naranjo A, Ding X, Zhang F, Hibbitts E, Kennedy R Clin Cancer Res. 2023; 29(8):1546-1556.

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The application and value of radiotherapy at the primary site in patients with high-risk neuroblastoma.

Wei Z, Li J, Jin Y, Liu Y, Wang P, Cao Y Br J Radiol. 2022; 95(1134):20211086.

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Genetic Alterations and Resectability Predict Outcome in Patients with Neuroblastoma Assigned to High-Risk Solely by Amplification.

Berthold F, Ernst A, Ackermann S, Bartenhagen C, Christiansen H, Hero B Cancers (Basel). 2021; 13(17).

PMID: 34503173 PMC: 8430929. DOI: 10.3390/cancers13174360.


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