Pediatric Hepatoblastoma. Complete Surgery and Ultrasound Monitoring

Abstract

Hepatoblastoma is the most common primary liver tumor of childhood. Most hepato­blastoma cases are sporadic, but others are associated with inherited conditions, sugges­ting the importance of genetic abnormalities in the pathogenesis and progression of this disease. A diverse range of cytogenetic altera­tions have been reported, as significant gains of genetic material, with various differences in the number and type of alterations between the different histologic components of hepatoblastoma. They could represent diagnostic marker, they may play a role in the tumorigenesis of hepatoblastoma and might become potential target for molecular therapeutics. An elevated serum alpha-fetoprotein is useful both at diagnosis of hepatoblastoma and in disease monitoring, by determining the success of surgical resection and/or response to adjuvant chemotherapy. Imaging has a significant role in the evaluation of hepatoblastoma, offering data about localization, resectability, and follow-up. Ultrasonography is the best initial imaging investigation of a child with a liver mass, allowing real-time investigation of the tumour. Complete surgical resection together with pre- and post­operative chemotherapy are the mainstay of treatment of hepatoblastoma. The potential down-staging effect of neoadjuvant chemotherapy on hepatoblastoma might facilitate remission and convert unresectable tumors into operable ones. Radical resection can be obtained either conventionally by partial hepatectomy or with orthotopic liver transplant. Complete hepatectomy with living donor liver transplantation provides optimal surgical treatment in unresectable stage III and initial stage IV disease confined to the liver at resection. Referral to a transplant center during the first cycles of chemotherapy appears to offer the best opportunity for long-term survival.