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Clinical Significance of MYCN Amplification and Ploidy in Favorable-Stage Neuroblastoma: A Report From the Children's Oncology Group

Jennifer Schneiderman, Wendy B. London, Garrett M. Brodeur, Robert P. Castleberry, A. Thomas Look, Susan L. Cohn

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.