Purpose: To examine the clinicopathological characteristics and prognosis of pediatric patients with malignant pancreatic tumors in a population-based cohort.
Methods: The Surveillance, Epidemiology, and End Results (SEER) database was utilized to identify all pediatric patients with malignant pancreatic tumors, diagnosed between 1973 and 2013. Kaplan-Meier analysis was performed to determine median and five-year overall survival (OS) rates. Univariate survival analysis was executed using the log-rank test. Cox proportional hazards model was used to identify variables independently associated with mortality.
Results: A total of 114 patients with pancreatic malignancies were identified. Median patient age was 16years and the majority of patients were white (64%) females (61.4%). The most prevalent histologic subtype was neuroendocrine tumors (35.1%), whereas pancreatoblastoma was more common during the first decade of life (P<0.001). Distant metastases were noted in 41.7% of the patients, while 33.3% and 25% had localized and regional disease respectively. Five-year OS rates were 77%, 66.4% and 64.8% for patients with pancreatoblastoma, neuroendocrine and epithelial tumors respectively. No death was observed in the solid pseudopapillary tumor group. Only history of having cancer-directed surgery (CDS) was significantly associated with lower overall mortality (HR: 5.1, 95% CI: 2.1, 12.4).
Conclusion: Pancreatic malignancies are rare in children. Their prognosis is variable and only CDS was independently associated with superior survival.
Evidence rating/classification: Prognosis study, Level II.
Keywords: Pancreatic neuroendocrine tumors; Pancreatic tumors; Pancreatoblastoma; Pediatric patients; SEER database; Solid pseudopapillary tumors.
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