Pancreatoblastoma of the pancreatic head with portal tumor embolism in a 2-year-old boy

A 2-year-old boy visited the hospital with a complaint of grayish white stool. A pancreatic tumor was found on investigation. The tumor was located at the head of the pancreas, and the common bile duct was completely occluded by the tumor and surrounding lymph nodes. A tumor embolism in the portal vein was observed. Development of accessory blood circulation associated with tumor embolization in the portal vein was observed. Tumor biopsy and biliary drainage tube placement were performed. The tumor was diagnosed as pancreatoblastoma. Pylorus-preserving pancreaticoduodenectomy was performed after three courses of chemotherapy. For the portal vein tumor plug, the portal vein was opened, and as much of the tumor plug was removed as possible. Anastomosis of the pancreas and small intestine was performed using the pancreatic duct insertion method, and the pancreatic juice was completely discharged from the body. After the operation, it was suspected that there was a small tumor remnant in the portal vein. Postoperative high-dose chemotherapy with peripheral blood stem cell transplantation and proton beam therapy were performed as additional treatments. Currently, the patient has been alive for 7 years without recurrence.