As no standard chemotherapy exists for pancreatoblastoma (PB), surgical resection is the most effective way of achieving complete remission.Case Report:
A 3-year-old girl with PB causing portal vein obstruction was referred to our hospital. Because of the portal vein involvement, she initially received 5 cycles of preoperative chemotherapy after biopsy, consisting of cyclophosphamide, vincristine, pirarubicin, and cisplatin. After chemotherapy, she underwent distal pancreatectomy, and the tumor was resected completely. She has been disease free for 4 years since the operation.Conclusions:
Even in cases of advanced PB, complete surgical resection with appropriate chemotherapy can lead to complete remission.