• Contemporary Analysis of Solid Pseudopapillary Neoplasms in Children Versus Adults

    Ioannis A Ziogas, Kristine S Corkum, Andrii Khomiak, Nicholas R Schmoke, Nicholas G Cost, Denis Bensard, Richard D Schulick, Marco Del Chiaro, Benedetto Mungo, Jonathan P Roach
    J Surg Res. 2025 Oct 6:315:224-230. doi: 10.1016/j.jss.2025.09.025. Online ahead of print.

    Abstract

    Introduction: Solid pseudopapillary neoplasm (SPN) comprises 2% of pancreatic tumors. We aimed to examine the clinical characteristics, management, and outcomes of SPN in children and adults using US-based national cancer registry data. 

     Methods: In this retrospective National Cancer Database analysis, children (<18 y) and adults (≥18 y) with SPN were included (2004-2020). 

     Results: A total of 1325 patients (231 children, 1094 adults) with SPN were identified. There were no differences between groups regarding tumor size, metastasis, or stage. Most patients in both groups underwent surgical treatment (96.5% versus 92.1%, P = 0.056), whereas a smaller proportion of children received chemotherapy compared to adults (0.9% versus 4.2%, P = 0.01). The 10-year overall survival rate was 99.1% in children versus 88.0% in adults. In multivariable Cox regression, when adjusted for tumor location and receipt of chemotherapy and radiation, adulthood (hazard ratio [HR]: 5.32, 95% confidence interval [95% CI]: 1.26-22.49, P = 0.02), Charlson-Deyo score ≥2 (HR: 3.04, 95% CI: 1.21-7.63, P = 0.02) compared to 0, stage II (HR: 2.48, 95% CI: 1.27-4.82, P = 0.008), stage III (HR: 6.82, 95% CI: 1.50-31.02, P = 0.01), and stage IV (HR: 5.94, 95% CI: 2.49-14.16, P < 0.001) compared to stage I were associated with an increased risk of mortality, whereas Hispanic compared to White patients (HR: 0.44, 95% CI: 0.21-0.95, P = 0.04) and surgically treated patients had a decreased risk of mortality (HR: 0.10, 95% CI: 0.05-0.21, P < 0.001). 

     Conclusions: Despite similar stage presentation, children with SPN exhibit prolonged survival compared to adults. Most patients underwent surgical treatment, whereas children were less likely to undergo chemotherapy than adults.