Primary Site of Neuroblastoma Tied to Differences in Biologic, Clinical Characteristics, Including Survival
In a study reported in Journal of Clinical Oncology, Vo et al in the International Neuroblastoma Risk Group Project found significant differences in clinical and biologic features of neuroblastoma according to primary tumor site, including poorer survival for adrenal sites of primary disease and better survival for thoracic sites of primary disease.
The study included data from 8,369 children in the International Neuroblastoma Risk Group database who were aged < 21 years and were diagnosed with neuroblastoma or ganglioneuroblastoma of known primary site between 1990 and 2002.
Biologic Features
Among all patients, 47% had adrenal, 24% abdominal/retroperitoneal, 15% thoracic, 3% pelvic, 3% neck, and 8% other primary tumor sites. All 16 evaluated clinical and biologic features showed significant differences across all six primary site categories (P < .001 for all comparisons). Features that exhibited > 10% discrepancy between sites were stage IV disease, MYCN amplification, elevated ferritin, elevated lactate dehydrogenase, and segmental chromosomal aberrations, with all being significantly more common in adrenal vs nonadrenal tumors (P < .001). After controlling for age, stage, and histologic grade, MYCN amplification was significantly more common in adrenal vs nonadrenal tumors (adjusted odds ratio [OR] = 2.09, P < .001) and significantly less common in thoracic vs nonthoracic tumors (adjusted OR = 0.20, P < .001).
Event-Free Survival and Overall Survival
On unadjusted analysis 5-year event-free survival and overall survival differed significantly according to primary site (P < .001 for both), with rates of 56% and 62% for adrenal, 64% and 72% for abdominal/retroperitoneal, 79% and 90% for neck, 80% and 88% for thoracic, 81% and 91% for pelvic, and 63% and 70% for other primary sites.
On Cox proportional hazards analysis adjusting for age, MYCN status, and stage, event-free survival remained significantly better in patients with thoracic vs adrenal tumors (adjusted hazard ratio [HR] = 0.76, P < .001), and overall survival remained significantly better in patients with thoracic (adjusted HR = 0.65, P < .001) or neck (adjusted HR = 0.54, P = .029) tumors vs those with adrenal tumors. Both event-free survival (adjusted HR = 1.13, P = .008) and overall survival (adjusted HR = 1.17, P = .003) were significantly poorer in patients with adrenal vs nonadrenal primary tumors, and both event-free survival (HR = 0.79, P = .003) and overall survival (adjusted HR = 0.68, P < .001) were significantly better in patients with thoracic vs nonthoracic tumors.
The investigators concluded: “Clinical and biologic features show important differences by [neuroblastoma] 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.”
Steven G. DuBois, MD, of University of California, San Francisco School of Medicine, is the corresponding author for the Journal of Clinical Oncology article.
The study was supported by the Cancer Research UK Institute of Cancer Research, National Institute for Health Research Royal Marsden/ICR Biomedical Research Centre, Alex’s Lemonade Stand Foundation, Frank A. Campini Foundation, Edward Conner Fund, and Dougherty Foundation. The authors reported no conflicts of interest.
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