Abstract
Introduction Malignant neuroendocrine tumors (NETs) are rare solid tumors infrequently seen in
children. This study examines current incidence trends and outcomes for children with
NETs.
Materials and Methods The Surveillance Epidemiology and End Results registry was examined for patients
between birth and 19 years of age between 1973 and 2005 with NETs.
Results Overall, 481 patients were identified with malignant NETs. Tumors were classified
as malignant carcinoid (n = 189, 39%), central nervous system tumors (n = 127, 26%), medullary thyroid carcinoma (n = 88, 18%), neuroendocrine carcinoma (n = 50, 10%), pheochromocytoma (n = 17, 3.5%), and paraganglioma (n = 7, 1.5%). Children less than 5 years of age had worse 5-year survival ( < 50 vs. > 76%
if older, p = 0.009). Blacks had lower 5-year survival compared with whites (61 vs. 81%, p = 0.001). Tumor locations with favorable prognosis included the appendix, lung, and
thyroid ( > 95% 5-year survival), whereas soft tissue and ovary location were associated
with poor survival (p < 0.001). In multivariate analysis, distant disease at diagnosis (odds ratio [OR]
14.9; 95% confidence interval [CI], 6.9 to 32.1) and black race (OR 3.3; 95% CI, 1.4
to 7.4) were independent predictors of death.
Conclusion Whites have the highest incidence of pediatric NETs. Colon/rectum, appendix, or thyroid
tumor location portends favorable prognosis. Black children and those less than 5
years of age had poorer prognosis.
Keywords
neuroendocrine tumors - outcomes research - pediatric oncology - survival
