Of 825 pediatric malignancies diagnosed at our institution between 2005 and 2012, 13 (1.6%) had primary liver tumors. The median age was 4 years (2 mo -15 yrs), 9 males. In 12 cases (92.3%), a malignant primary was present of which hepatoblastoma was the commonest, seen in 8(67%) cases. Hepatocellular carcinoma (HCC) was diagnosed in 3(25%) and undifferentiated embryonal sarcoma(UES) in 1(8%). Mesenchymal hamartoma was identified in 1 child. For hepatoblastoma and HCC risk stratification was done according to PRETEXT criteria and SIOPEL-3 chemotherapy protocol was used [2]. Serum alfafetoprotein (AFP) was measured in all cases and serially monitored.

Only eight children (61.5%) opted for therapy (6 hepatoblastoma, 1 UES and 1 mesenchymal hamartoma) of which 5 are alive and well at a median follow-up of 30 months. Two with PRETEXT IV disease underwent orthotopic liver transplant (OLT). Five had complete excision of the involved lobe/s and 1 with harmatoma had partial excision. Three relapsed and died (2 hepatoblastoma and 1 UES). Of the relapsed hepatoblastoma patients, 1 was high risk and had undergone OLT. The other did not show expected decline in his AFP levels post complete surgical resection and later relapsed in lungs and bone. There were no deaths in peri-operative period and none due to sepsis or cardiotoxicity. Five (30.7%) abandoned therapy soon after diagnosis (HCC-3 and Hepatoblastoma-2).

AFP monitoring is vital in management of hepatic tumors. Both very high and low levels of AFP are associated with a poor outcome. Failure of AFP to decline to age appropriate levels with therapy is associated with a high risk of relapse/disease progression [3]. Interestingly, in two patients of hepatoblastoma we found a maternal history of colon cancer. Association of hepatic tumors with inherited syndromes such as familial adenomatous polyposis is well known [4] and must be searched for. Treatment abandonment is a major hurdle in improving outcome of pediatric liver tumors. However, reasonably good outcomes can be achieved if patient comply with therapy.

1. Arora RS. Outcomes of hepatoblastoma in the Indian context. Indian Pediatr. 2012;49:307-9.

2. Zsíros J, Maibach R, Shafford E, Brugieres L, Brock P, Czauderna P, et al. Successful treatment of childhood high-risk hepatoblastoma with dose-intensive multiagent chemotherapy and surgery: final results of the SIOPEL-3HR study. J Clin Oncol. 2010;28:2584-90.

3. Van Tornout JM, Buckley JD, Quinn JJ, Feusner JH, Krailo MD, King DR, et al. Timing and magnitude of decline in alpha-fetoprotein levels in treated children with unresectable or metastatic hepatoblastoma are predictors of outcome: a report from the Children’s Cancer Group. J Clin Oncol. 1997;15:1190-7.