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Primary tumor site affected clinical features, outcomes in pediatric neuroblastoma
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Tumor features and outcomes varied considerably by primary tumor site among children with neuroblastoma, according to study results.
Kieuhoa T. Vo, MD, of Benioff Children’s Hospital and University of California, and colleagues used the International Neuroblastoma Risk Group database to evaluate data on 8,369 patients aged younger than 21 years who were diagnosed with neuroblastoma or ganglio neuroblastoma between 1990 and 2002. Primary tumor sites were adrenal (47.3%), abdominal/retroperitoneal (23.7%), thoracic (15.1%), pelvic (3%), neck (2.7%) and other (7.9%).
Researchers evaluated clinical and biologic features, EFS and OS on all patients and compared outcomes based on primary tumor sites.
Vo and colleagues observed statistically significant differences in all evaluated clinical and biologic variables based on primary tumor site.
Results showed several features were >10% discrepant between sites. They were stage IV disease, MYCN amplification, elevated ferritin, elevated lactate dehydrogenase and segmental chromosomal aberrations. All of those features were more common in patients with adrenal tumors than nonadrenal tumors (P<.001).
After researchers controlled for age, stage and histologic grade, they determined adrenal tumors were more likely than nonadrenal tumors to have MYCN amplification (adjusted OR=2.09; 95% CI, 1.67-2.63). Also, thoracic tumors were less likely than nonthoracic tumors to have MYCN amplification (adjusted OR=0.2; 95% CI, 0.11-0.39).
In a model that evaluated EFS in all six primary tumor sites, only children with thoracic tumors remained at decreased risk for an event (adjusted HR=0.76; 95% CI, 0.64 to 0.89) compared with children in the adrenal-tumor reference group.
Analysis of a similar model for OS showed patients with thoracic primary tumor sites (adjusted HR=0.65; 95% CI, 0.52-0.8) or neck primary tumor sites (adjusted HR=0.54; 95% CI, 0.34-0.94) demonstrated a lower risk for death than those in the adrenal-tumor reference group.
Vo and colleagues determined OS and EFS differed significantly based on primary tumor site (P<.001 for both).
Children with adrenal tumors demonstrated a significantly higher risk for (HR=1.13; 95% CI, 1.03-1.23) and death (adjusted HR=1.17; 95% CI, 1.05-1.29) than those who had nonadrenal tumors.
Children with thoracic tumors had a significantly lower risk for events (HR=0.79; 95% CI, 0.67-0.92) and death (adjusted HR=0.68; 95% CI, 0.56-0.84) than those with nonthoracic tumors.
“Our results suggest that there is something distinctive about the tumor in these specific sites of origin that leads to or reflects different biology and clinical behavior,” Vo and colleagues wrote. “Further study of the developmental biology of the neural crest and sympathetic nervous system may elucidate the etiology for these observed differences. Likewise, additional efforts should be directed at elucidating the disordered mechanisms of embryonal tumorigenesis in neuroblastoma.”
Disclosure: The researchers report no relevant financial disclosures.
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