Purpose: Pancreatic tumors are rare in children and limited data are available regarding incidence, treatment, and outcomes. We aim to describe patient and tumor characteristics and to report on survival of these diseases.
Methods: Children with pancreatic tumors were queried from the National Cancer Database (2004-2014). The association between treatment and hazard of death was assessed using Kaplan-Meier method and Cox regression model.
Results: We identified 109 children with pancreatic tumors; 52% were male and median age at diagnosis was 14 years. Tumors were distributed as follows: pseudopapillary neoplasm (30%), endocrine tumors (27%), pancreatoblastoma (16%), pancreatic adenocarcinoma (16%), sarcoma (6%) and neuroblastoma (5%). Seventy-nine patients underwent surgery, of which 76% achieved R0 resection. Most patients (85%) had lymph nodes examined, of which 22% had positive nodes. Five-year overall survival by tumor histology was 95% (pseudopapillary neoplasm), 75% (neuroblastoma), 70% (pancreatoblastoma), 51% (endocrine tumors), 43% (sarcoma), and 34% (adenocarcinoma). On multivariable analysis, surgical resection was the strongest predictor of survival (HR 0.26, 95% CI 0.10-0.68, p < 0.01).
Conclusion: Overall survival of children with pancreatic tumors is grim, with varying survival rates among different tumors. Surgical resection is associated with improved long-term survival.
Keywords: Children; Pancreatic tumors; Pediatric; Survival; Treatment.