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Comparative Study
. 2011 Jan;56(1):50-7.
doi: 10.1002/pbc.22559.

Incidence, survival, and prevalence of neuroendocrine tumors versus neuroblastoma in children and young adults: nine standard SEER registries, 1975-2006

Pournima Navalkele  1 , M Sue O'Dorisio, Thomas M O'Dorisio, Gideon K D Zamba, Charles F Lynch
Affiliations
Comparative Study

Incidence, survival, and prevalence of neuroendocrine tumors versus neuroblastoma in children and young adults: nine standard SEER registries, 1975-2006

Pournima Navalkele et al. Pediatr Blood Cancer. 2011 Jan.

Abstract

Background: The incidence, survival, and prevalence of neuroendocrine tumors (NETs) in children were determined as a first step in improving diagnosis and therapy. Outcomes were compared with neuroblastoma, a pediatric malignancy that shares several biomarkers.

Methods: Incidence rates, observed survival rates and 31-year limited duration prevalence counts were obtained from SEER*Stat for diagnosis years 1975 to 2006. These rates were compared between and within NETs and neuroblastoma for demographic and tumor-related variables from nine standard SEER registries for ages 0-29 years. Multivariate Cox regression was performed to identify prognostic factors for survival in NETs.

Results: The number of NETs was 1,073 compared to 1,664 neuroblastomas. The most common NET sites were lung, breast, and appendix. NET 5-year observed survival rates increased from 83% between 1975 and 1979 to 84% for the 2000-2006 period, while analogous neuroblastoma survival rates steadily increased from 45-73%. Five-year observed survival was less than 30% in females with NETs of the cervix and ovary. The estimated 31-year limited duration prevalence for NETs as of January 1, 2006 in the U.S. population was 7,724 compared to 9,960 for neuroblastomas. Age-adjusted multivariate Cox Regression demonstrated small cell histology, primary location in the breast, and distant stage as major predictors of decreased survival.

Conclusions: While survivorship has significantly increased for neuroblastoma, those diagnosed with NETs have shown no increase in survival during this 31-year period. NETs constitute an unrecognized cancer threat to children and young adults comparable to neuroblastoma in both number of affected persons and disease severity.

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Conflict of interest statement

Conflict of Interest Statement: None of above authors has any relevant conflict of interest to disclose.

Figures

Figure 1
Figure 1
Distribution of 1073 malignant neuroendocrine tumors by common primary sites, ages 0–29 years, 9 Standard SEER Registries, 1975–2006
See this image and copyright information in PMC

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