Aggressive surgical and chemotherapeutic treatment of advanced pancreatoblastoma associated with tumor thrombus in portal vein.

A 3-year-old boy had pancreatoblastoma arising in the pancreatic head and body, with metastasis in the mesenteric lymph nodes, tumor thrombus in the portal vein, and a small metastatic lesion in the left lung. After preoperative chemotherapy, aggressive surgical treatment including resection and reconstruction of the portal and superior mesenteric veins was performed. Postoperative chemotherapy was followed by high-dose chemotherapy and rescue with peripheral blood stem cell transplantation, and he has now been well for 6 years. This is the first surviving pediatric case of advanced pancreatoblastoma treated by aggressive surgical therapy including reconstruction of the portal vein and high-dose chemotherapy followed by peripheral blood stem cell transplantation.