Pancreatitis in Pediatric Patients with Pancreatic Solid Pseudopapillary Neoplasms: A Single Center Experience

Infographic · Jan 2026 · 1 min read

In brief

Single-center study of 10 female pediatric patients with solid pseudopapillary neoplasms found that all who developed post-operative pancreatitis had either hereditary gene mutations (CFTR variants, PRSS1 duplication) or anatomic abnormalities (pancreas divisum). Findings suggest genetic and anatomic evaluation prior to resection may help predict post-operative outcomes in this rare pediatric population.

All pediatric SPN patients who developed post-op pancreatitis had pancreatic head lesions and genetic/anatomic risk factors (CFTR variants or divisum).
Pre-operative genetic testing and anatomic evaluation may predict post-operative ARP/CP risk in pediatric SPN resection candidates.
Three of ten patients required completion pancreatectomy with islet autotransplantation, highlighting need for multidisciplinary planning.
EUS was performed in only half of cases; standardized pre-op imaging protocols may improve surgical planning and outcomes.
Pediatric SPN resection requires coordinated care between surgery, genetics, and gastroenterology to optimize long-term pancreatic function.

Written by the GCMD Library team from the infographic.

The infographic uses a teal and pink color scheme with illustrated icons including a calendar, DNA helix, test tubes, pancreas diagram, and a patient in bed. Content is organized in distinct sections with colored boxes highlighting key findings and risk factors. The layout flows from top to bottom with study details, findings, and clinical implications.

Stephen J Hartman, Nicolas Noriega, Raphael M Parrado, Emily Vore , Andrew Trout , David Vitale , Maisam Abu-El-Haija , Juan P Gurria

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.

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Pancreatitis in Pediatric Patients w/ Pancreatic Solid Pseudopapillary Neoplasms (SPNs) | Single-center Retrospective Case Series | 2010-2023 | 10 children undergoing SPN resection | Median age at diagnosis = 15 yrs old | 100% female | 50% underwent preoperative endoscopic ultrasound | Post-op pancreatitis only occurred in patients with pancreatic head SPNs PLUS | Anatomic risk factors: Pancreas divisum w/ pancreaticojejunostomy stricture | Genetic risk factors: Heterozygous CFTR pathogenic variant | SPN resection may unmask pancreatitis susceptibility. | Clinical implications: | Consider preoperative genetic testing evaluate pancreatic anatomy before resection multidisciplinary care is key | Conclusion: Genetic and anatomic predisposition is closely linked to post-resection pancreatitis. Pre-op evaluation may help predict risk. | https://pubmed.ncbi.nlm.nih.gov/41448454/ | Hartman SJ et. al. | Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, USA | Journal of Pediatric Surgery | @LizzyPAC8 | @globalcastmd | @StayCurrentMD | Cincinnati Children's