TY - JOUR
T1 - Malignant neuroendocrine tumors
T2 - Incidence and outcomes in pediatric patients
AU - Allan, Bassan
AU - Davis, James
AU - Perez, Eduardo
AU - Lew, John
AU - Sola, Juan
PY - 2013/3/1
Y1 - 2013/3/1
N2 - 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.
AB - 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.
KW - neuroendocrine tumors
KW - outcomes research
KW - pediatric oncology
KW - survival
UR - http://www.scopus.com/inward/record.url?scp=84884590896&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84884590896&partnerID=8YFLogxK
U2 - 10.1055/s-0033-1333643
DO - 10.1055/s-0033-1333643
M3 - Article
C2 - 23444065
AN - SCOPUS:84884590896
VL - 23
SP - 394
EP - 399
JO - European Journal of Pediatric Surgery
JF - European Journal of Pediatric Surgery
SN - 0939-7248
IS - 5
ER -