Malignant neuroendocrine tumors: Incidence and outcomes in pediatric patients

Bassan Allan, James Davis, Eduardo Perez, John Lew, Juan E Sola

Research output: Contribution to journalArticle

18 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)394-399
Number of pages6
JournalEuropean Journal of Pediatric Surgery
Volume23
Issue number5
DOIs
StatePublished - Mar 1 2013

Fingerprint

Neuroendocrine Tumors
Pediatrics
Incidence
Survival
Appendix
Neoplasms
Thyroid Gland
Odds Ratio
Confidence Intervals
Neuroendocrine Carcinoma
Paraganglioma
Central Nervous System Neoplasms
Carcinoid Tumor
Pheochromocytoma
Rectum
Registries
Ovary
Colon
Epidemiology
Multivariate Analysis

Keywords

  • neuroendocrine tumors
  • outcomes research
  • pediatric oncology
  • survival

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

Cite this

Malignant neuroendocrine tumors : Incidence and outcomes in pediatric patients. / Allan, Bassan; Davis, James; Perez, Eduardo; Lew, John; Sola, Juan E.

In: European Journal of Pediatric Surgery, Vol. 23, No. 5, 01.03.2013, p. 394-399.

Research output: Contribution to journalArticle

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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.",
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