Prolonged survival after resection of pancreatoblastoma and synchronous liver metastases in an adult.

摘要

Pancreatoblastoma is an uncommon pediatric neoplasm with distinct acinar and squamoid cell differentiation. Pancreatoblastoma is exceedingly rare in adults with only ten reported cases. Pancreatoblastoma in adults has a poor prognosis and no survival without recurrence exceeding 30 months has been reported. We report the first adult case of pancreatoblastoma revealed by gastric bleeding due to segmental hypertension. On computed tomography scan, the tumor appeared lobulated and extended from the splenic hilum to the portal vein. Two hypervascular centimetric hepatic metastases were observed in segments III and VII. The patient was operated and a distal pancreatectomy with splenectomy associated with two hepatic wedge resections was performed. The diagnosis of pancreatoblastoma was made on immunohistochemical examination. The patient received 6 cycles of adjuvant therapy. After three years of follow-up, the patient was well with no sign of recurrence on computed tomography scan. This case suggests that in the presence of pancreatic tumor of unknown origin, aggressive management including complete surgical resection and adjuvant chemotherapy should be attempted even in the presence of synchronous liver metastases.