Originally published as JCO Early Release 10.1200/JCO.2008.17.9184 on December 1 2008

This Article Full Text Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Bagatell, R. Articles by Cohn, S. L. Search for Related Content PubMed PubMed Citation Articles by Bagatell, R. Articles by Cohn, S. L. Social Bookmarking                            What's this?

Significance of MYCN Amplification in International Neuroblastoma Staging System Stage 1 and 2 Neuroblastoma: A Report From the International Neuroblastoma Risk Group Database

Rochelle Bagatell, Maja Beck-Popovic, Wendy B. London, Yang Zhang, Andrew D.J. Pearson, Katherine K. Matthay, Tom Monclair, Peter F. Ambros, Susan L. Cohn

From the University of Arizona Health Sciences Center, Tucson, AZ; University of Florida, Gainesville, FL; University of California, San Francisco, CA; The University of Chicago, Chicago, IL; University Hospital Centre Hospitalier Vaudois, Lausanne, Switzerland; Children's Oncology Group Statistics and Data Center, Institute of Cancer Research/Royal Marsden Hospital, Sutton, United Kingdom; Rikshospitalet University Hospital, Oslo, Norway; and Children's Cancer Research Institute, St Anna Kinderkrebforschung, Vienna, Austria

Corresponding author: Rochelle Bagatell, MD, Division of Oncology, Children's Hospital of Philadelphia, 3516 Civic Center Blvd, Philadelphia, PA 19104; e-mail: bagatellr{at}email.chop.edu

Purpose Treatment of patients with localized neuroblastoma with unfavorable biologic features is controversial. To evaluate the outcome of children with low-stage MYCN-amplified neuroblastoma and develop a rational treatment strategy, data from the International Neuroblastoma Risk Group (INRG) database were analyzed.

Patients and Methods The database is comprised of 8,800 patients. Of these, 2,660 patients (30%) had low-stage (International Neuroblastoma Staging System stages 1 and 2) neuroblastoma, known MYCN status, and available follow-up data. Eighty-seven of these patients (3%) had MYCN amplified tumors.

Results Patients with MYCN-amplified, low-stage tumors had less favorable event-free survival (EFS) and overall survival (OS) than did patients with nonamplified tumors (53% ± 8% and 72% ± 7% v 90% ± 1% and 98% ± 1%, respectively). EFS and OS were statistically significantly higher for patients whose tumors were hyperdiploid rather than diploid (EFS, 82% ± 20% v 37% ± 21%; P = .0069; OS, 94% ± 11% v 54% ± 15%; P = .0056, respectively). No other variable had prognostic significance. Initial treatment consisted of surgery alone for 29 (33%) of 87 patients. Details of additional therapy were unknown for 14 patients. Twenty-two patients (25%) underwent surgery and moderate-intensity chemotherapy; another 22 underwent surgery, intensive chemotherapy, and radiation therapy. Nine of the latter 22 underwent stem cell transplantation. Survival in patients who received transplantation did not differ from survival in those who did not receive transplantation.

Conclusion Among patients with low-stage, MYCN-amplified neuroblastoma, outcomes of patients with hyperdiploid tumors were statistically, significantly better than those with diploid tumors. The data suggest that tumor cell ploidy could potentially be used to identify candidates for reductions in therapy. Further study of MYCN-amplified, low-stage neuroblastoma is warranted.

published online ahead of print at www.jco.org on December 1, 2008.

Supported in part by the William Guy Forbeck Research Foundation, the Little Heroes Cancer Research Fund, and the Caitlin Robb Foundation.

Presented in part at the 13th Advances in Neuroblastoma Research Conference, May 21–24, 2008, Chiba, Japan.

Authors’ disclosures of potential conflicts of interest and author contributions are found at the end of this article.

CiteULike    Complore    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this?