Malignant Liver Tumors in South African Children: A National Audit
Malignant liver tumors (mostly hepatoblastoma [HB] and hepatocellular carcinoma [HCC]) are uncommon, representing 0.5%–2% of childhood malignancies worldwide. The pattern of liver tumors appears to differ in Southern Africa as a result of infectious factors (e.g., hepatitis B/retroviral disease (HIV). This study aimed to assess recent changes in the prevalence and surgical management of liver tumors in South African children.
Data were obtained from the tumor registry and pediatric oncology units in South African hospitals to audit and review the epidemiology, treatment, and outcome of malignant hepatic tumors in South African children.
Malignant primary hepatic tumors were reported in 274 children (ages 0–14 years) from 1988 through June 2006. Of these 134 (48%) had HB; 77 (27%) had HCC (9 [3%] fibrolamellar subtype); 38 (13%), vascular tumors; and 17 (6%), liver sarcomas. In a further 8 patients (3%) other tumors included lymphoma and endodermal sinus tumor. Vascular tumors included hemangioendotheliomas (12), and there were 5 malignant tumors in children with HIV, including 1 angiosarcoma and 13 Kaposi sarcoma-like tumors. Hepatoblastoma occurred at a mean age of 1.47 years, and none were encountered in patients > 4 years of age. Hepatocellular carcinoma mostly occurred in the older patients (mean age: 10.48 years), but 6% presented in patients < 8 years of age (10 months, 2, 2.6, 5, 5, and 6 years). Hepatic sarcoma occurred at a mean age of 7.66 years and had a female predominance (M:F ratio: 0.4). The relative HCC prevalence (male predominant: hepatitis B related) was reflected in the low HB:HCC (1.67) ratio. However, a significant decrease in HCC was attributed to the effect of hepatitis B inoculation. There appeared to be an increase in the incidence of vascular tumors, presumably the result of an increase in Kaposi-like sarcoma in retrovirus-positive patients. The surgical resection rate was low because most patients presented late, with advanced disease. Survival was 11% and 52% for HB and HCC, respectively, and was related to chemotherapeutic response and complete surgical resection.
Liver tumors appear to have a different epidemiological pattern in South Africa. The observed increased HCC prevalence appears to be decreasing with hepatitis B vaccination. Retroviral disease does not yet appear to have a major influence on the distribution of liver tumors in South Africa, although it possibly affects the vascular tumor prevalence.
- 16.Exelby P, Filler R, Grosfeld JL (1974) Liver tumors in children in the particular reference to hepatoblastoma and hepatocellular carcinoma: American Academy of Pediatrics Surgical survey system. J Pediatr Surg 10:329–337Google Scholar
- 22.von Schweinitz D, Burger D, Weinel P et al (1994) Therapy of malignant liver tumors in childhood: an intermediate report of the HB–89 multicentre study of the GPOH. Med Pediatr Oncol 23:170–287Google Scholar
- 25.Munoz N, Bosch FX (1987) Epidemiology of hepatocellular carcinoma. In: Okuda K, Ishgak K (eds) Neoplasms of the Liver, Springer-Verlag, Tokyo, pp 3–19Google Scholar
- 26.Okuda K, Okuda H (1999) Malignant tumors. In: Bircher J, Benhamou J-P, McIntyeare N et al (eds) Oxford Textbook of Clinical Hepatology, Oxford Medical Publishers, New York, pp 1491–1530Google Scholar
- 27.Stocker JT, Husain AN, Dehner LP et al (2001) The liver, gallbladder and biliary tract. In: Stocker JT, Dehner LP, (eds) Pediatric Pathology, Williams and Wilkins, Philadelphia, Lipincott, pp 705–797Google Scholar
- 29.von Schweinitz D, Byeard DJ, Hecker H et al (1997) Efficiency and toxicity of ifosfamide, cisplatin and doxorubicin in the treatment of childhood hepatoblastoma. Study Committee of the Cooperative Paediatric Liver Tumor Study HB89 of the German Society for Paediatric Oncology and Haematology. Eur J Cancer 33:1243–1249CrossRefGoogle Scholar