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Eur J Pediatr Surg. 2013 Oct;23(5):394-9. doi: 10.1055/s-0033-1333643. Epub 2013 Feb 26.

Malignant neuroendocrine tumors: incidence and outcomes in pediatric patients.

Author information

1
Department of Surgery, University of Miami, Miami, Florida, United States.

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.

PMID:
23444065
DOI:
10.1055/s-0033-1333643
[Indexed for MEDLINE]

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