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Management and outcome of stage 3 neuroblastoma.


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.

SUBMITTER: Modak S 

PROVIDER: S-EPMC3727624 | biostudies-literature | 2009 Jan

REPOSITORIES: biostudies-literature

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Publications

Management and outcome of stage 3 neuroblastoma.

Modak Shakeel S   Kushner Brian H BH   LaQuaglia Michael P MP   Kramer Kim K   Cheung Nai-Kong V NK  

European journal of cancer (Oxford, England : 1990) 20081106 1


<h4>Purpose</h4>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.<h4>Methods</h4>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 (O  ...[more]

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