Solid Pseudopapillary Neoplasm of the Pancreas: Clinical-Radiological-Pathological Characteristics of Four Pediatric Cases

Abstract

Solid Pseudopapillary Neoplasm (SPN) of the pancreas represents 1-3 % of all exocrine pancreatic tumors and is uncommon in children. We report four pediatric patients with SPN where each patient posed a unique diagnostic and therapeutic challenge. We describe these four cases with detailed clinical-radiological-pathological correlations. All patients are female, with a median age 13.5 years. Two patients presented with abdominal pain, one with jaundice and one with an incidental pancreatic mass on abdominal CT scan. Radiological studies included abdominal ultrasound, CT scan and MRI of abdomen. Pancreaticoduodenectomy was performed in three patients and laparoscopic distal pancreatectomy in one patient. Mean tumor size was 4.5 cm (ranged from 1.9 to 11.5 cm). All SPNs were benign on histological exam. One patient developed pancreatic insufficiency post-surgery. No tumor recurrence was observed over a mean follow up period of 1 year. We conclude that diagnosis of SPN in pediatric population can be challenging due to non-specific clinical findings, and surgical removal of the tumor is usually required for definitive histologic diagnosis and treatment. Most tumors are benign and recurrence is very rare.