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Journal of Clinical Oncology, Vol 26, No 6 (February 20), 2008: pp. 913-918 © 2008 American Society of Clinical Oncology. 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
From the Children's Memorial Hospital and the Department of Pediatrics, Northwestern University, Feinberg School of Medicine; Department of Pediatrics, The University of Chicago and Comer Children's Hospital, Chicago, IL; University of Florida, Children's Oncology Group Statistics and Data Center, Gainesville, FL; The Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania, Philadelphia, PA; Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL; and Dana-Farber Cancer Institute and Children's Hospital, Harvard Medical School, Boston, MA Corresponding author: Susan L. Cohn, MD, Section of Pediatric Hematology/Oncology, University of Chicago, 5841 Maryland Ave, MC 4060, Rm N114, Chicago, IL 60637; e-mail: scohn{at}peds.bsd.uchicago.edu 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. Supported by the Children's Oncology Group Statistics and Data Center Grants No. U10CA29139, CA25408, CA 98543, and CA 30969; the Neuroblastoma Children's Cancer Society; Friends for Steven Pediatric Cancer Research Fund; the Elise Anderson Neuroblastoma Research Fund, Neuroblastoma Kids; and National Institutes of Health Grants No. R01 NS049814 and R01 CA039771. Presented in part at the Advances in Neuroblastoma (ANR) Meeting in Los Angeles, CA, May 17-20, 2006. Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.
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Copyright © 2008 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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