Solid pseudopapillary neoplasm of the pancreas in children

To characterize the clinical features of solid pseudopapillary neoplasm of the pancreas (SPN) in pediatric patients. Clinical data from five pediatric patients diagnosed with pancreatic SPN and treated at Shanxi Children’s Hospital between January 1, 2018, and July 1, 2025, were retrospectively reviewed. Data included clinical presentation, imaging findings, surgical management, postoperative complications, and outcomes. Relevant literature was also reviewed. Of the five patients, three were female and two were male (age range: 9–13 years). Three were identified incidentally, two presented with a 2-day history of abdominal pain and vomiting. No patient had jaundice. Preoperative neuron-specific enolase was elevated in two patients; all other markers were normal. Tumors were located in the pancreatic head ( n = 2), pancreatic neck ( n = 1), and body and tail ( n = 2). Contrast-enhanced computed tomography demonstrated the “floating cloud sign” in two cases and calcification in two cases. Magnetic resonance imaging (MRI) was performed in three patients and showed heterogeneous mixed-signal mass lesions in all, without bile duct or main pancreatic duct involvement. One patient had MRI findings of portal hypertension. Tumor enucleation was performed in the patient with a pancreatic head SPN and in the patient with a pancreatic neck SPN. The pancreatic neck enucleation patient developed a postoperative pancreatic fistula refractory to conservative management and required internal drainage of a pancreatic pseudocyst into the jejunum. The two patients with body and tail SPNs underwent spleen-preserving distal pancreatectomy; the patient with a pancreatic head SPN underwent tumor enucleation combined with pancreatoenterostomy. All patients recovered satisfactorily following surgery and remained asymptomatic at follow-up. Pediatric SPN is a low-grade malignant neoplasm predominantly affecting young females but occurring in males as well. A conservative surgical strategy prioritizing pancreatic function preservation yields favorable outcomes in children.