Abstract

Background: Hepatoblastoma (HBL) is the most common primary hepatic tumour in children. The aim of the study was retrospective analysis of the type of management and its results in children with HBL in our centre for over the last 19 years.
Material/Methods: Between 1990 and 2008 there were 44 children with  hepatoblastoma treated in our centre. All patients were treated with various protocols of chemotherapy. In 32 children partial resection of liver was  performed, in 9 with unresectable tumour liver - transplantation. Among them there were 2 pts with pulmonary metastases cleared by chemotherapy, and 2 with local tumour recurrence after resection done elsewhere. In three children with inoperable disease (unresectable tumour and pulmonary and bone metastases) only palliative chemotherapy was administered.
Results: There were 9 deaths among children treated with liver resection  (28.1%), and 4 deaths in the group treated with liver transplantation (44.4%), but only 1 in 7 patients with local recurrence would was excluded (14.3%). Overall survival rate among all 44 children with HBL was 64%. Follow-up of living patients who underwent resection ranges from 1 to 190 months (mean 80
months). All deaths were due to recurrence of the disease. Follow-up of living patients who were transplanted ranges from 22 to 78 months (mean 42 months). The cause of death in OLTx group was recurrence of the disease (in metastatic form) in 3 patients and PTLD in 1 patient. All 3 children who were not qualified for surgical treatment died of hepatoblastoma.
Conclusions: With the improved selection for the two types of surgical treatment: partial liver resection or total hepatectomy and liver transplantation excellent results can be obtained in children with hepatoblastoma limited to the liver (including pts with cleared pulmonary metastases). Prognosis for the patients with recurrent disease or with inoperable tumour remains poor despite improved chemotherapy.

Keywords: Liver Transplantation