Pancreatoblastoma(PB)is one of the exocrine pancreatic tumors and the most common malignant pancreatic tumor in young children.Patients can present with abdominal distension,pain,fatigue,vomiting and failure to thrive...Pancreatoblastoma(PB)is one of the exocrine pancreatic tumors and the most common malignant pancreatic tumor in young children.Patients can present with abdominal distension,pain,fatigue,vomiting and failure to thrive.Exocrine pancreatic tumors usually affect patients between 1 and 8 years,with a median age of 5 years.There is a slight preponderance in males and those of Asian descent[1].PB is considered to be embryonic in origin with moderately raised serum alpha-fetoprotein(AFP)levels[2].Axial imaging is necessary to assess other organ involvement,a common complication in adult patients,but diagnosis is confirmed on tissue histology[3].Complete surgical resection is the treatment of choice,if achievable.Indications for neoadjuvant systemic chemotherapy include large tumors that involve adjacent major blood vessels or other organs and metastatic disease[1].Herein we report a pediatric case of PB with liver metastases who underwent liver transplantation for metastatic liver disease.展开更多
文摘Pancreatoblastoma(PB)is one of the exocrine pancreatic tumors and the most common malignant pancreatic tumor in young children.Patients can present with abdominal distension,pain,fatigue,vomiting and failure to thrive.Exocrine pancreatic tumors usually affect patients between 1 and 8 years,with a median age of 5 years.There is a slight preponderance in males and those of Asian descent[1].PB is considered to be embryonic in origin with moderately raised serum alpha-fetoprotein(AFP)levels[2].Axial imaging is necessary to assess other organ involvement,a common complication in adult patients,but diagnosis is confirmed on tissue histology[3].Complete surgical resection is the treatment of choice,if achievable.Indications for neoadjuvant systemic chemotherapy include large tumors that involve adjacent major blood vessels or other organs and metastatic disease[1].Herein we report a pediatric case of PB with liver metastases who underwent liver transplantation for metastatic liver disease.
