Pancreatoblastoma with portal vein involvement in a child: A case report

Abstract

Pancreatoblastoma is a rare pancreatic tumor in children. Its biologic behavior is aggressive with frequent local invasion, recurrence, and metastasis, for which there has been no standard treatment regimen.

A 13-year old Japanese girl had pancreatoblastoma arising from the pancreas head and body and invading the portal vein. The tumor was resected with pancreaticoduodenectomy combined with reconstruction of the portal and splenic vein using native left common iliac vein graft, followed by chemotherapy. The tumor recurred in the liver and periportal lymph node but was treated with surgical resection and chemotherapy followed by autologous peripheral blood stem cell transplantation. The reconstructed portal vein has been patent with no sign of stricture for 3.5 years after the first operation. She has been well with a second complete remission for 10 months.

Complete surgical resection has been considered for long-term survival of patients with pancreatoblastoma. Portal venous involvement is not a contraindication of surgical resection of pancreatic tumors and may be aggressively treated by pancreaticoduodenectomy with portal venous reconstruction using a venous conduit. Autologous peripheral blood stem cell transplantation has been used in only two other pediatric cases in the literature for treating pancreatoblastoma. Although our follow-up period is short, aggressive surgical and chemotherapeutic regimen may hold therapeutic promise for managing advanced or relapsed pancreatoblastoma in children.