title: Malignant neuroendocrine tumors: incidence and outcomes in pediatric patients.
abstract: BACKGROUND
  Malignant neuroendocrine tumors (NETs) are rare solid tumors infrequently seen in children. This study examines current incidence trends and outcomes for children with NETs.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors: Department of Surgery, University of Miami, Miami, Florida, United States.
authors: Bassan
authors: B
authors: Allan
authors: James
authors: J
authors: Davis
authors: Eduardo
authors: E
authors: Perez
authors: John
authors: J
authors: Lew
authors: Juan
authors: J
authors: Sola
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D008297:Male; D015999:Multivariate Analysis; D018358:Neuroendocrine Tumors / Q000175:diagnosis / Q000453:epidemiology* / Q000401:mortality; D011379:Prognosis; D012307:Risk Factors; D018426:SEER Program; D015996:Survival Rate; D014481:United States / Q000453:epidemiology; D055815:Young Adult