Eur J Pediatr Surg 2013; 23(05): 394-399
DOI: 10.1055/s-0033-1333643
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Malignant Neuroendocrine Tumors: Incidence and Outcomes in Pediatric Patients

Bassan Allan
1   Department of Surgery, University of Miami, Miami, Florida, United States
,
James Davis
1   Department of Surgery, University of Miami, Miami, Florida, United States
,
Eduardo Perez
1   Department of Surgery, University of Miami, Miami, Florida, United States
,
John Lew
1   Department of Surgery, University of Miami, Miami, Florida, United States
,
Juan Sola
1   Department of Surgery, University of Miami, Miami, Florida, United States
› Author Affiliations
Further Information

Publication History

25 September 2012

08 December 2012

Publication Date:
26 February 2013 (online)

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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.