Clinical, Biologic, and Prognostic Differences on the Basis of Primary Tumor Site in Neuroblastoma: A Report From the International Neuroblastoma Risk Group Project

• DOI: 10.1200/jco.2014.56.1621
• Publication Date: 2014-08-26T05:08:06Z
• Authors: Kieuhoa T. Vo; Katherine K. Matthay; John Neuhaus; Wendy B. London; Barbara Hero; Peter F. Ambros; Akira Nakagawara; Doug Miniati; Kate Wheeler; Andrew D.J. Pearson; Susan L. Cohn; Steven G. DuBois
• Keywords: Primary tumor

Purpose Neuroblastoma (NB) is a heterogeneous tumor arising from sympathetic tissues. The impact of primary site in influencing the heterogeneity NB remains unclear. Patients and Methods Children younger than age 21 years diagnosed with or ganglioneuroblastoma between 1990 2002 known were identified International Risk Group database. Data compared sites respect to clinical biologic features, as well event-free survival (EFS) overall (OS). Results Among 8,369 children, 47% had adrenal tumors. All evaluated variables differed statistically sites. features that > 10% discrepant stage 4 disease, MYCN amplification, elevated ferritin, lactate dehydrogenase, segmental chromosomal aberrations, all which more frequent versus nonadrenal tumors (P < .001). Adrenal likely (adjusted odds ratio, 2.09; 95% CI, 1.67 2.63; P .001) thoracic less nonthoracic 0.20; 0.11 0.39; have amplification after controlling for age, stage, histologic grade. EFS OS significantly according .001 both comparisons). After status, patients higher risk events (hazard 1.13 tumors; 1.03 1.23; = .008), lower (HR, 0.79 nonthoracic; 0.67 0.92; .003). Conclusion Clinical show important differences by site, associated inferior superior survival, respectively. Future studies will need investigate origin these differences.