Solid Pseudo-Papillary Neoplasm of Pancreas with Venous Invasion in A 9 Year Old Girl: A Case Report and Literature Review

Abstract

Solid Pseudo-papillary neoplasm of the pancreas (SPN) is a rare entity. It represents 0.2-2.7% of all pancreatic cancers. Predominantly occurs in young females in the second to third decades of life. The etiology of SPN involves mutations in the gene that encodes beta-catenin. SPNs are typically indolent tumors, usually confined to the pancreas. We report a case of SPN in a 9-year-old female presented with intermittent abdominal pain for four months. Imaging studies demonstrated a 2.8 cm mass in the tail of the pancreas. The patient underwent a distal pancreatectomy. Pathological evaluation was diagnostic for SPN in the tail of the pancreas. Our case is distinct because of the young age of the patient, peripancreatic soft tissue, perineural, and lymphovascular invasion. The tumor cells exhibited cytoplasmic and nuclear immunoreactivity for beta-catenin and progesterone receptors.