Aim: Pancreatic tumors in children are exceedingly rare and therefore present significant diagnostic and therapeutic challenges for pediatric surgeons. We reviewed our institutional experience with these rare entities.
Methods: We conducted a retrospective single-institution study of all pediatric pancreatic tumors treated over a 15-year period (2009–2024). Data collected included clinical symptoms at presentation, diagnostic evaluations, types of surgical interventions, and patient outcomes.
Results: Eighteen patients were identified (thirteen female, five male). The tumor types included solid pseudopapillary neoplasm (SPN) (n = 13), low-grade neuroendocrine tumor (n = 2), pancreatoblastoma (n = 1), adenocarcinoma of the ampulla of Vater (n = 1), and metastasis of the extrahepatic component of hepatoblastoma (n = 1). Surgical resections performed were pancreatoduodenectomy (n = 8), open distal pancreatectomy (n = 3), laparoscopic distal pancreatectomy (n = 3), duodenum-preserving pancreatectomy (n = 2), enucleation (n = 1), and combined pancreatoduodenectomy with right hepatectomy (n = 1). Post-surgical complications included pancreatic leak and fistula in the patient who underwent enucleation, and late pancreatojejunostomy stenosis in a patient following pancreatoduodenectomy. The patients with adenocarcinoma and hepatoblastoma died to metastatic disease 18 and 11 months postoperatively, respectively, despite achieving tumor-free resection margins after pancreatoduodenectomy and right hepatectomy. The remaining patients are alive with no evidence of recurrence at a mean follow-up of 53 months.
Conclusions: Complete surgical resection remains the only potential cure for pediatric patients with malignant pancreatic tumors; however, survival remains poor in those with locally advanced disease, even when R0 resection is achieved. SPN is the most common pediatric pancreatic tumor, characterized by low-grade malignant potential and a favorable prognosis following complete surgical resection.
Keywords: pancreatic tumors, solid pseudopapillary neoplasm, pancreatectomy, hepatectomy
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