Hepatocellular Carcinoma: Referral to a Transplantation Unit

Abstract

Aim Hepatocellular carcinoma (HCC), although being infrequent, is the second-most common primary hepatic malignancy in children, after hepatoblastoma (HB). The prognosis is very poor. We present our series of children with HCC referred to our transplant unit to be assessed as candidates for liver transplantation (LT).

Methods A retrospective review of HCCs referred to our transplant unit in the past 20 years (1994–2015) was performed. Age at diagnosis, disease-free survival, location of recurrence, initial treatment, secondary treatment, and mortality were noted.

Main Results Ten patients (8 boys, 2 girls) met the inclusion criteria. Median age at diagnosis was 11.5 years (0.5–14). HCC was associated with tyrosinemia in two patients, while the tumor developed in absence of previous liver disease in eight. Seven children attempted tumor resection earlier elsewhere. LT was not considered suitable in six patients due to extrahepatic tumor extension and finally it was performed in four (two with tyrosinemia and two with “de novo” HCC). Only one of the transplants was primary, and the other three were performed as rescue therapy. After 78 (66–90) months of follow-up, the two patients with tyrosinemia remain alive and disease free, while the other two had distant relapses, 35 and 37 months after LT, respectively, and finally died due to tumor progression.

Conclusions HCC is a rare, very aggressive tumor in children who has a very poor prognosis. Our results suggest the need for new strategies. Early referral of all cases to highly specialized centers with a liver transplant unit and perhaps a more liberal use of LT, even for selected, apparently resectable cases, are possible options.

Keywords

hepatocellular carcinoma - liver transplantation - pediatrics

• References

• 1 Czauderna P, Mackinlay G, Perilongo G , et al; Liver Tumors Study Group of the International Society of Pediatric Oncology. Hepatocellular carcinoma in children: results of the first prospective study of the International Society of Pediatric Oncology group. J Clin Oncol 2002; 20 (12) 2798-2804
• 2 Allan BJ, Wang B, Davis JS , et al. A review of 218 pediatric cases of hepatocellular carcinoma. J Pediatr Surg 2014; 49 (1) 166-171 , discussion 171
• 3 Fuchs J, Seitz G, Handgretinger R, Warmann SW. Extended hepatic resection in advanced hepatoblastoma. Front Biosci (Elite Ed) 2012; 4: 462-469
• 4 Slotta JE, Kollmar O, Ellenrieder V, Ghadimi BM, Homayounfar K. Hepatocellular carcinoma: surgeon's view on latest findings and future perspectives. World J Hepatol 2015; 7 (9) 1168-1183
• 5 van Spronsen FJ, Thomasse Y, Smit GP , et al. Hereditary tyrosinemia type I: a new clinical classification with difference in prognosis on dietary treatment. Hepatology 1994; 20 (5) 1187-1191
• 6 Katzenstein HM, Krailo MD, Malogolowkin MH , et al. Hepatocellular carcinoma in children and adolescents: results from the Pediatric Oncology Group and the Children's Cancer Group intergroup study. J Clin Oncol 2002; 20 (12) 2789-2797
• 7 McAteer JP, Goldin AB, Healey PJ, Gow KW. Surgical treatment of primary liver tumors in children: outcomes analysis of resection and transplantation in the SEER database. Pediatr Transplant 2013; 17 (8) 744-750
• 8 Chan SC, Fan ST. Selection of patients of hepatocellular carcinoma beyond the Milan criteria for liver transplantation. Hepatobiliary Surg Nutr 2013; 2 (2) 84-88
• 9 Liang W, Wu L, Ling X , et al. Living donor liver transplantation versus deceased donor liver transplantation for hepatocellular carcinoma: a meta-analysis. Liver Transpl 2012; 18 (10) 1226-1236
• 10 Weeda VB, Murawski M, McCabe AJ , et al. Fibrolamellar variant of hepatocellular carcinoma does not have a better survival than conventional hepatocellular carcinoma—results and treatment recommendations from the Childhood Liver Tumour Strategy Group (SIOPEL) experience. Eur J Cancer 2013; 49 (12) 2698-2704
• 11 Murawski M, Weeda VB, Maibach R , et al. Hepatocellular carcinoma in children: does modified platinum- and doxorubicin-based chemotherapy increase tumor resectability and change outcome? lessons learned from the SIOPEL 2 and 3 studies. J Clin Oncol 2016; 34 (10) 1050-1056
• 12 Otte JB, Meyers R. PLUTO first report. Pediatr Transplant 2010; 14 (7) 830-835