Pancreatic Head Sparing Surgery for Solid Pseudopapillary Tumors in Patients with Agenesis of the Dorsal Pancreas

Abstract

Introduction: This study aimed to clarify the feasibility and justification of pancreatic head sparing (PHS) enucleation for patients with agenesis of the dorsal pancreas (ADP) associated with a solid pseudopapillary tumor (SPT). Method: Data of the SPT patients with and without ADP, including clinical presentations, surgical options, and surgical and survival outcomes, were recruited for comparison. Results: A total of 31 patients with SPTs were included, 3 of whom displayed ADP and underwent PHS enucleation. Surgical complications were comparable between the groups. Overall, the 5-year and 10-year disease-free survival rates were 100% and 90%, respectively. The 20-year and 25-year overall survival rates were 100% and 66.7%, respectively. Only one patient (3.2%) developed tumor recurrence 7.3 years after pancreatectomy for an SPT with lymph node involvement, and the patient survived 24.5 years after the initial operation. No tumor recurrence occurred in any patient with ADP after PHS enucleation. Conclusions: The association between tumorigenesis and ADP suggests that every congenital ADP patient should be observed for the early detection of pancreatic neoplasia. PHS enucleation seems to be feasible and justifiable for SPT patients with ADP in terms of surgical and survival outcomes, and this approach could be recommended to avoid pancreatic insufficiency.