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
