Hepatoblastoma in children

Hepatoblastoma is the most common primary liver tumour in children. Complete surgical removal is the treatment of choice for cure; however, in most cases the tumour is unresectable because of its extensive hepatic involvement. Nineteen pediatric cases (11 boys, 8 girls) with ages ranging from three months to 17 years were referred for management to our clinic from 1982 until 2000. All but three suffered from abdominal distention. The other frequent complaints were abdominal mass, anorexia, fatigue, abdominal pain and fever. Physical examination revealed enlarged liver in all patients. In addition to laboratory studies, they were pre-operatively examined by ultrasonography and, in recent cases, computed tomography was also used. Serum alpha-fetoprotein levels were found to be elevated in all patients. In thirteen cases, hepatic resections (10 lobectomies, 2 trisegmentectomies, 1 segmentectomy) were performed. In six children only liver biopsies could be done because of the huge tumour size. However, in three of them the tumours were excised at the second laparotomy, but only one patient survived. All of the patients - except two who were lost in the early postoperative period - received chemotherapy whether the tumour was excised or biopsied. In this series the mortality rate was found to be very high (91%) in the 1980s, and more reasonable (50%) in the 1990s, with an overall mortality rate of 73 per cent. This result might be explained with late referral and advanced stage at diagnosis. In addition, we speculate that a combination of improved chemotherapy and technical advances in anesthesia and hepatic resection caused the obvious differences in the survival rates between the two periods.