Solid Pseudopapillary Neoplasm of the Pancreas in Children and Adolescents: the Expert Recommendations

Background and Aims: Solid pseudopapillary neoplasm of the pancreas (SPN) is a rare low-grade malignant exocrine pancreatic tumour, mostly discovered during the second decade of life in females. Due to the very low incidence in children, current management is based on retrospective studies. This European Standard Clinical Practice (ESCP) from the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) aims to provide harmonized recommendations for the diagnosis, treatment, and long-term management of paediatric SPN. Methods: The EXPeRT group decided on the formation of a working group to develop diagnostic and therapeutic recommendations. These were finalized after a literature review and on the base of the Consensus Conference Standard Operating Procedure Methodology, in which levels of evidence and degrees of recommendation are considered for each suggested therapeutic intervention. Results: SPN has a favourable prognosis with a post-operative mortality of 1-2%. Abdominal pain is the main symptom at diagnosis, followed by abdominal mass discovery or pancreatitis. The laboratory work-up should assess both the pancreatic function and differential diagnoses. An abdominal MRI should be preferred to CT-scan. A biopsy is not recommended except if the diagnosis is doubtful. Surgery is the cornerstone treatment and needs en-bloc resection, with microscopically safe margins and no spillage. Duodenopancreatecomy may be preferred for headlocated primary, splenopancreatectomy or left pancreatectomy for tail and central pancreatectomy for body primary. Enucleation is a valuable option. Significant post-operative morbidity can occur due to exocrine and/or endocrine pancreatic insufficiency. Relapse should be primarily operated on. In case of distant locations, focal therapies may be discussed. Conclusions: Despite its rarity, SPN is the most frequent pancreatic tumour in childhood and has a very good prognosis, providing microscopically complete surgical excision is achieved. Implementation of these recommendations aims to optimize early diagnosis, harmonize therapy, improve treatment outcomes, and enhance long-term follow-up strategies for affected patients.