Background: Though rare, solid pseudopapillary neoplasms (SPN) are one of the most common, low-grade, malignant pediatric pancreatic neoplasms in children and young adults and are associated with a good prognosis. This study seeks to describe characteristics, genetics and outcomes of patients who underwent SPN resection.
Methods: A single-center retrospective series of pediatric patients who underwent SPN resection from 2010-2023. SPN location, surgical intervention, and rates of acute recurrent and chronic pancreatitis (ARP, CP) were summarized. Patients who developed ARP/CP underwent pancreatitis-specific genetic testing.
Results: Ten patients were included, all of whom were female. Median age at diagnosis was 15.0 years (13.8, 18.3). Half (n=5) underwent preoperative endoscopic ultrasound (EUS). Five patients underwent pancreaticoduodenectomy, the remainder underwent partial pancreatectomy. Of the three patients who went on to develop post-operative ARP/CP all had SPN lesions of the pancreatic head. Individual histories were notable for an established risk alle heterozygous CFTR variant, a heterozygous CFTR variant of unknown significance, and pancreas divisum with pancreaticojejunostomy (PJ) stricture respectively. One patient initially presented with acute pancreatitis with known PRSS1 MLPA duplication, but did not progress to ARP/CP. Three patients required completion pancreatectomy with islet autotransplantation.
Conclusions: These data represent a unique pediatric patient population requiring coordinated, multi-disciplinary care. Given the identification of either hereditary gene mutations or pancreas divisum in all patients who developed ARP/CP after SPN resection, genetic and anatomic evaluation prior to resection might provide greater understanding of post-operative outcomes after resection. Further evaluation is needed among pediatric patients with SPN.
