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Clinical, biologic, and prognostic differences on the basis of primary tumor site in neuroblastoma: a report from the international neuroblastoma risk group project
- Source :
- Journal of clinical oncology : official journal of the American Society of Clinical Oncology, vol 32, iss 28
- Publication Year :
- 2014
-
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.
- Subjects :
- Oncology
Cancer Research
Pathology
Databases, Factual
International Cooperation
Adrenal Gland Neoplasms
Disease
Kaplan-Meier Estimate
Neuroblastoma
Risk Factors
Stage (cooking)
Child
Ganglioneuroblastoma
Cancer
Pelvic Neoplasms
Pediatric
Oncogene Proteins
N-Myc Proto-Oncogene Protein
Nuclear Proteins
ORIGINAL REPORTS
Prognosis
Primary tumor
Head and Neck Neoplasms
Child, Preschool
medicine.medical_specialty
Pediatric Cancer
Adrenal Gland Neoplasm
Clinical Sciences
Oncology and Carcinogenesis
Risk Assessment
Databases
Rare Diseases
Clinical Research
Internal medicine
medicine
Humans
Oncology & Carcinogenesis
Preschool
Factual
Neoplasm Staging
Proportional Hazards Models
Chromosome Aberrations
L-Lactate Dehydrogenase
business.industry
Proportional hazards model
Neurosciences
Gene Amplification
Infant
Thoracic Neoplasms
medicine.disease
Logistic Models
Ferritins
business
Subjects
Details
- ISSN :
- 15277755
- Volume :
- 32
- Issue :
- 28
- Database :
- OpenAIRE
- Journal :
- Journal of clinical oncology : official journal of the American Society of Clinical Oncology
- Accession number :
- edsair.doi.dedup.....ea787dce9c1128b93a73604d5e565b91