Outcomes in Paediatric Solid Pseudopapillary Neoplasms of the Pancreas

Objective: To investigate outcomes and risk factors for recurrence and pancreatic insufficiency in paediatric Solid Pseudopapillary Neoplasm (SPN) of the pancreas. Background: SPN is a low-grade malignant pancreatic tumour and uncommon in children. Recommended treatment is complete surgical resection. Long-term problems include recurrence and pancreatic insufficiency. The low incidence in children complicates consolidation of high-quality data. Paediatric-specific guideline recommendations on follow-up are missing. Methods: Systematic review of Ovid/Medline/Embase/Web-of-Science databases from inception-2024, investigating outcomes after paediatric SPN resection. Meta-analysis, univariate and multivariate analysis were performed. Results: 778 articles were identified and 37 articles (701 patients) analysed. Median age was 14 years [IQR12;15], 584 (83.2%) patients were female. Median follow-up was 52.5 months [IQR 34;71]. Recurrence developed in 40 (5.7%) patients; at a median of41 months post-operatively [IQR 36;48]. Multivariate analysis identified positive resection margin (OR 48.1, P <0.001) and large tumour size (OR 1.44, P <0.001) associated with recurrence. Univariate analysis also identified neurovascular invasion and positive lymph node status as riskfactors. Endocrine dysfunction developed in 10/157 patients (median 14 mo [IQR 14;98] after surgery). Exocrine dysfunction developed in 9/157 patients: (median 15 mo [IQR 6;15]). Four (0.57%) patients died during reported follow-up, two with identified disease recurrence. Conclusion: This meta-analysis of paediatric patients with SPN demonstrates a significantly increased risk of tumour recurrence in patients with larger tumours and positive resection margins, at a median of 3.4 years after surgery. This highlights the need for systematic long-term follow-up of children with SPN. International collaborative studies are warranted to improve paediatric-specific data.