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.