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J Clin Oncol. 2014 Oct 1;32(28):3169-76. doi: 10.1200/JCO.2014.56.1621. Epub 2014 Aug 25.

Clinical, biologic, and prognostic differences on the basis of primary tumor site in neuroblastoma: a report from the international neuroblastoma risk group project.

Author information

1
Kieuhoa T. Vo, Katherine K. Matthay, John Neuhaus, and Steven G. DuBois, Benioff Children's Hospital and University of California, San Francisco, San Francisco; Doug Miniati, Kaiser Permanente Medical Center, Roseville, CA; Wendy B. London, Children's Oncology Group Statistics and Data Center and Dana-Farber Children's Hospital Cancer Center, Boston, MA; Barbara Hero, Children's Hospital, University of Cologne, Köln, Germany; Peter F. Ambros, Children's Cancer Research Institute, St Anne Kinderkrebsforschung, Vienna, Austria; Akira Nakagawara, Chiba Cancer Center Research Institute and Chiba University, Chiba, Japan; Kate Wheeler, Oxford Children's Hospital, Oxford; Andrew D.J. Pearson, Institute of Cancer Research and Royal Marsden Hospital, Surrey, United Kingdom; Susan L. Cohn, The University of Chicago, Chicago, IL.
2
Kieuhoa T. Vo, Katherine K. Matthay, John Neuhaus, and Steven G. DuBois, Benioff Children's Hospital and University of California, San Francisco, San Francisco; Doug Miniati, Kaiser Permanente Medical Center, Roseville, CA; Wendy B. London, Children's Oncology Group Statistics and Data Center and Dana-Farber Children's Hospital Cancer Center, Boston, MA; Barbara Hero, Children's Hospital, University of Cologne, Köln, Germany; Peter F. Ambros, Children's Cancer Research Institute, St Anne Kinderkrebsforschung, Vienna, Austria; Akira Nakagawara, Chiba Cancer Center Research Institute and Chiba University, Chiba, Japan; Kate Wheeler, Oxford Children's Hospital, Oxford; Andrew D.J. Pearson, Institute of Cancer Research and Royal Marsden Hospital, Surrey, United Kingdom; Susan L. Cohn, The University of Chicago, Chicago, IL. duboiss@peds.ucsf.edu.

Abstract

PURPOSE:

Neuroblastoma (NB) is a heterogeneous tumor arising from sympathetic tissues. The impact of primary tumor site in influencing the heterogeneity of NB remains unclear.

PATIENTS AND METHODS:

Children younger than age 21 years diagnosed with NB or ganglioneuroblastoma between 1990 and 2002 and with known primary site were identified from the International Neuroblastoma Risk Group database. Data were compared between sites with respect to clinical and biologic features, as well as event-free survival (EFS) and overall survival (OS).

RESULTS:

Among 8,369 children, 47% had adrenal tumors. All evaluated clinical and biologic variables differed statistically between primary sites. The features that were > 10% discrepant between sites were stage 4 disease, MYCN amplification, elevated ferritin, elevated lactate dehydrogenase, and segmental chromosomal aberrations, all of which were more frequent in adrenal versus nonadrenal tumors (P < .001). Adrenal tumors were more likely than nonadrenal tumors (adjusted odds ratio, 2.09; 95% CI, 1.67 to 2.63; P < .001) and thoracic tumors were less likely than nonthoracic tumors (adjusted odds ratio, 0.20; 95% CI, 0.11 to 0.39; P < .001) to have MYCN amplification after controlling for age, stage, and histologic grade. EFS and OS differed significantly according to the primary site (P < .001 for both comparisons). After controlling for age, MYCN status, and stage, patients with adrenal tumors had higher risk for events (hazard ratio, 1.13 compared with nonadrenal tumors; 95% CI, 1.03 to 1.23; P = .008), and patients with thoracic tumors had lower risk for events (HR, 0.79 compared with nonthoracic; 95% CI, 0.67 to 0.92; P = .003).

CONCLUSION:

Clinical and biologic features show important differences by NB primary site, with adrenal and thoracic sites associated with inferior and superior survival, respectively. Future studies will need to investigate the biologic origin of these differences.

PMID:
25154816
PMCID:
PMC4171360
DOI:
10.1200/JCO.2014.56.1621
[Indexed for MEDLINE]
Free PMC Article

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