HEPATOCELLULAR-CARCINOMA IN CHILDREN

Hepatocellular carcinoma (HCC) occurs more frequently in subsaharan Af rica and the Orient than in other geographic regions, but remains an u ncommon tumour of childhood. We review six children with HCC (mean age 13 years) treated by the paediatric oncology unit at Tygerberg Hospit al in Cape Town over an 8-year period (1953-1990), Patients presented with epigastric and right upper quadrant discomfort and hepatomegaly. The hepatitis B serum antigen (HbsAg) was positive in three patients; serum alpha-fetoprotein (AFP) levels were markedly elevated in three ( range 100-453,000 mu g/l). Age and sex did not differ significantly an d all patients initially had irresectable advanced-stage tumours. Morp hologically, three were highly malignant adult-type pleomorphic HCCs, two were differentiated tumours, and one a fibrolamellar subtype. The mean 2-year survival was 33% and the 5-year survival 16.6%. The biolog ical behaviour and response to treatment of the tumours varied. Wherea s three patients had a poor response to therapy, two with poorly-diffe rentiated tumours, negative HbsAg, and normal serum AFP levels respond ed to doxorubicin/cisplatinum chemotherapy. This facilitated radical s urgical excision. One patient of this group has survived for more than 75 months following surgical resection and remains well, HCC remains an uncommon tumour of childhood with a high modality. Aggressive chemo therapeutic regimes in combination surgical resection may lead to impr oved survival in some cases, Prevention of hepatitis E remains a prior ity.