Pediatric Solid Pseudopapillary Neoplasm of the Pancreas

Abstract

ABSTRACT Background: Solid pseudopapillary neoplasm (SPN) of the pancreas in children is a rare tumor with low malignant potential. Some tumors, however, behave aggressively. There is very little literature on managing these variants, especially in children. We share our experience of managing large and recurrent SPN and explore the clinicopathological findings correlating to the risk of recurrence. Methods: This is a retrospective study of children treated for SPN between 2012 and 2022 at a tertiary care center in India. The clinicopathological features and management strategies in these children were evaluated. Results: Sixteen children with SPN were treated during this period (88% of girls). The median age of presentation was 12 years (interquartile range [IQR]: 9–14). All children presented with abdominal pain. Computed tomography gave a definitive diagnosis in 81% of cases. The tumor predominantly involved the head of the pancreas (n = 9, 56%). Eight of nine children classified as high-grade (HG) malignant had a benign course. One child had a recurrence of the tumor 4 years after the initial resection and further recurrence on chemotherapy. She required radiation therapy in addition to reoperation following which she was disease free for 77 months. The overall median follow-up was 46 months (IQR: 18–72 months). Conclusion: Complete resection of the tumor provides a cure in most patients with SPN. Recurrent tumors require a multi-modality approach. Long-term survival is good. There is a need for clear definitions of the components within the WHO criteria for HG malignancy.