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. 2009 Jan;45(1):90-8.
doi: 10.1016/j.ejca.2008.09.016. Epub 2008 Nov 6.

Management and outcome of stage 3 neuroblastoma

Shakeel Modak  1 , Brian H Kushner, Michael P LaQuaglia, Kim Kramer, Nai-Kong V Cheung
Affiliations

Management and outcome of stage 3 neuroblastoma

Shakeel Modak et al. Eur J Cancer. 2009 Jan.

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.

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Conflict of interest statement

Conflicts of interest statement

None declared.

Figures

Fig. 1
Fig. 1
Memorial Sloan-Kettering Cancer Centre (MSKCC) algorithm for management of patients with International Neuroblastoma (NB) Staging System (INSS) stage 3 NB.
Fig. 2
Fig. 2
Distribution of patients with stage 3 NB.
Fig. 3
Fig. 3
Kaplan–Meier analysis for overall survival in patients with stage 3 NB: group A: patients with MYCN-non-amplified NB treated with surgical resection without chemotherapy; group B: MYCN-non-amplified NB patients treated with chemotherapy at non-MSKCC institutions prior to surgical resection and group C: patients with MYCN-amplified NB.
Fig. 4
Fig. 4
Kaplan–Meier analysis for event-free survival in patients with stage 3 NB.
See this image and copyright information in PMC

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