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J Clin Oncol. 2008 Feb 20;26(6):913-8. doi: 10.1200/JCO.2007.13.9493.

Clinical significance of MYCN amplification and ploidy in favorable-stage neuroblastoma: a report from the Children's Oncology Group.

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  • 1Children's Memorial Hospital, Department of Pediatrics, Northwestern University, Feinberg School of Medicine, Chicago, IL, USA.

Abstract

PURPOSE:

MYCN amplification is rarely detected in patients with favorable-stage neuroblastoma (NB). To determine the clinical significance of MYCN amplification in children with favorable-stage NB, we performed a retrospective review of data from the Pediatric Oncology Group (POG) biology study 9047.

PATIENTS AND METHODS:

MYCN status, tumor cell ploidy, treatment, and outcome of patients with stage A, B, or Ds NB, enrolled on POG 9047 between 1990 and 1999 were analyzed. Event-free survival (EFS) and overall (OS) survival rates were analyzed using the Kaplan-Meier method.

RESULTS:

Of the 1,667 patients enrolled on POG 9047, 643 had favorable-stage disease. Of these, follow-up data were available on 568 (34%) with stage A, B, or Ds disease and normal MYCN copy number, and 32 (1.9%) patients with MYCN-amplified, stage A, B, or Ds tumors. Within the cohort lacking MYCN amplification, the 7-year EFS and OS rates (+/- SE) were 91% +/- 1% and 96% +/- 1%, respectively. Patients with MYCN amplification had significantly worse EFS and OS (50% +/- 9% and 59% +/- 9%, respectively, P < .0001). Within the cohort of children with MYCN amplification, the 7-year EFS and OS rates were 80% +/- 10% and 87% +/- 9%, respectively for patients with hyperdiploid tumors and 25% +/- 11% and 38% +/- 12% for patients with diploid/hypodiploid NBs (P = .0063 and P = .0074, respectively).

CONCLUSION:

Tumor cell ploidy may be a clinically useful factor for prognostication and treatment stratification in children with MYCN-amplified, favorable-stage NB tumors.

PMID:
18281664
[PubMed - indexed for MEDLINE]
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