Pancreatic neuroendocrine carcinoma is extremely rare in pediatric population. Huge abdominal mass is one of the
most common presenting symptoms of malignant tumor in infants. Surgical resection is demonstrated as the best treatment for survival of neuroendocrine tumors. We presented a nine month-old infant with huge right abdominal mass. MRI revealed an abnormal, large well-defined mixed solid-cystic lesion at right retroperitoneal region, measured as 8.9x8.8x9.7 cm in size. We had performed a pyloric-preserving pancreaticoduodenectomy by using modified Blumgart’s pancreaticojejunostomy anastomosis. A duct-to-mucosa was constructed under internal pancreatic duct stent without suture by using a cut ETFE sheath of the SAFELET CATHTM 24G. The pathology revealed pancreatic neuroendocrine carcinoma with a Ki-67 index of 80%. Immunostaining for chromogranin A and synaptophysin were positive, whereas CK7 and CK20 were negative. Postoperative care was uneventful. He received combined etoposide and cisplatin for adjuvant chemotherapy. After chemotherapy, an MRI revealed no evidence of tumor recurrence. Pancreaticoduodenectomy is a feasible and safe surgical method for the radical treatment of PNEC in infant. Modified Blumgart’s pancreaticojejunostomy with duct-to-mucosa constructed under internal pancreatic duct stent without sutured is feasible for a very small pancreatic duct.

Keywords: Neuroendocrine tumor, Pancreaticoduodenectomy, Pancreas