title: Malignant pancreatic tumors: incidence and outcome in 58 pediatric patients.
abstract: OBJECTIVE
  The purpose of the study was to examine current incidence trends and outcomes for children with pancreatic malignancies.
  
  
  METHODS
  The Surveillance, Epidemiology, and End Results registry (1973-2004) was examined for pediatric patients with pancreatic malignancies (up to 19 years of age).
  
  
  RESULTS
  Malignant pancreatic neoplasms were identified in 58 patients. Females outnumbered males 1.9 to 1 (38 vs 20) for an age population-adjusted incidence of 0.021 and 0.015 per 100,000. Overall, 70% (n = 41) of patients were white. Asians had the highest incidence. Tumors were classified as exocrine (n = 31, 53.4%), endocrine (n = 19, 32.8%), or sarcomas (n = 5, 8.6%). Exocrine tumors included pancreatoblastoma (n = 10), solid-cystic tumor (SCT) (n = 10), ductal adenocarcinoma (DA) (n = 7), and acinar cell carcinoma (ACC) (n = 4). All SCTs and 80% of pancreatoblastomas were seen in females, whereas males had a higher incidence of DA 71% (P = .036). Ductal adenocarcinoma, SCT, acinar cell carcinoma, sarcomas, and endocrine tumors were more common in children older than 10 years, whereas pancreatoblastoma was more common in younger children (P = .045). Almost half of patients (n = 25) presented with distant metastasis; of these, 44% were endocrine tumors. Survival was significantly greater for female patients (P = .004) and for those who had surgery (P = .001) by both univariate and multivariate analysis. There was a significant difference in tumor type 15-year survival with DA having the worst (23%) and SCT the best (100%).
  
  
  CONCLUSIONS
  Pediatric pancreatic neoplasms are uncommon and carry a variable prognosis. Both female sex and surgery were independent predictors of improved survival.
authors.affiliation: DeWitt Daughtry Family Department of Surgery, University of Miami Miller School of Medicine, FL 33136, USA.
authors.forename: Eduardo A
authors.initials: EA
authors.lastname: Perez
authors.forename: Juan C
authors.initials: JC
authors.lastname: Gutierrez
authors.forename: Leonidas G
authors.initials: LG
authors.lastname: Koniaris
authors.forename: Holly L
authors.initials: HL
authors.lastname: Neville
authors.forename: William R
authors.initials: WR
authors.lastname: Thompson
authors.forename: Juan E
authors.initials: JE
authors.lastname: Sola
mesh_terms: D000293:Adolescent; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D010190:Pancreatic Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery*; D016016:Proportional Hazards Models; D012307:Risk Factors; D018426:SEER Program; D015996:Survival Rate; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology