Contemporary Analysis of Solid Pseudopapillary Neoplasms in Children Versus Adults

Ioannis A Ziogas1, Kristine S Corkum2, Andrii Khomiak3

  • 1Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, Colorado; Surgical Oncology Program, Children's Hospital Colorado, Aurora, Colorado; Division of Surgical Oncology, Department of Surgery, University of Colorado School of Medicine, Aurora, Colorado.

PubMed

Insights

Children with solid pseudopapillary neoplasm (SPN) have significantly better survival rates than adults, despite similar disease presentation. Surgery is the primary treatment for both groups, but children receive chemotherapy less often.

Area of Science:

  • Oncology
  • Pediatric Oncology
  • Surgical Oncology

Background:

  • Solid pseudopapillary neoplasm (SPN) represents a rare but distinct group of pancreatic tumors, accounting for approximately 2% of all pancreatic neoplasms.
  • Understanding the clinical nuances, management strategies, and long-term outcomes of SPN is crucial for optimizing patient care, particularly when comparing pediatric and adult populations.

Purpose of the Study:

  • To investigate and compare the clinical characteristics, treatment modalities, and survival outcomes of solid pseudopapillary neoplasm (SPN) in pediatric versus adult patients.
  • To identify prognostic factors influencing mortality risk in patients diagnosed with SPN.

Main Methods:

  • Retrospective analysis of the National Cancer Database (NCDB) from 2004 to 2020.
  • Inclusion criteria comprised patients diagnosed with SPN, stratified into pediatric (<18 years) and adult (≥18 years) cohorts.
  • Statistical analyses included comparisons of demographics, tumor characteristics, treatment received, and survival rates, utilizing multivariable Cox regression for prognostic factor identification.

Main Results:

  • A total of 1325 SPN cases were analyzed (231 children, 1094 adults), with no significant differences in tumor size, metastasis, or stage at presentation between the groups.
  • Surgical resection was the predominant treatment in both pediatric (96.5%) and adult (92.1%) cohorts; however, chemotherapy use was significantly lower in children (0.9% vs. 4.2%).
  • The 10-year overall survival rate was markedly higher in children (99.1%) compared to adults (88.0%). Adulthood, higher Charlson-Deyo score, advanced tumor stage (II-IV), and receipt of chemotherapy/radiation were associated with increased mortality risk, while Hispanic ethnicity and surgical treatment correlated with decreased mortality.

Conclusions:

  • Children with solid pseudopapillary neoplasm (SPN) demonstrate superior long-term survival outcomes compared to adults, even when diagnosed at similar disease stages.
  • While surgical intervention is standard for both age groups, the lower utilization of chemotherapy in pediatric SPN warrants further investigation.
  • Adulthood and advanced disease stage are significant predictors of mortality in SPN patients.
Abstract