Pediatric solid pseudopapillary neoplasm of the pancreas

Abstract

AbstractBackgroundSolid pseudopapillary neoplasm of the pancreas (SPN) in children is rare tumor with low malignant potential. Some tumors however behave aggressively. There is very little literature on the management of 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.MethodsThis is a retrospective study of children who were 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.Results16 children with SPN were treated during this period (88% girls). The median age of presentation was 12 years (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 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 is disease free for 77 months. The overall median follow-up was 46 months (IQR 18-72 months).ConclusionComplete resection of the tumor provides a cure in most patients with SPN. Recurrent tumors require a multi-modality approach. Long-term survival is good. Better prognostic criteria with immunohistochemistry are required to predict the behavior of these tumors as the WHO criteria for malignancy correlate poorly with clinical outcomes in childhood SPN.