Background:

Hepatocellular carcinoma (HCC) is an aggressive hepatic neoplasm that most commonly affects the adults. Nevertheless, children who are affected with biliary atresia, infantile cholestasis, glycogen storage diseases, and a wide array of cirrhotic diseases of the liver are predisposed to developing HCC. Most children have abdominal findings localized to the liver.

The 2 pathological subtypes are classic HCC and fibrolamellar carcinoma. Although surgical resection remains the mainstay of curative therapy, adjunctive chemotherapeutic and radiotherapeutic strategies are also helpful. The presenting signs and symptoms, epidemiology, biology, and current therapeutic strategies for HCC follow.

Pathophysiology: The pathophysiology of HCC is not understood clearly, but underlying liver dysfunction, especially cirrhosis, is a predisposing condition. Karyotypic abnormalities are not common. In adults, infectious or alcoholic cirrhosis and chronic hemochromatosis are highly associated with HCC. Although children and adolescents are unlikely to have chronic liver disease, congenital liver disorders increase the chance of developing HCC. These findings are suggestive of a multihit model of malignant transformation in hepatic tissue.

Frequency:

·         In the US: Primary liver tumors are uncommon in children and adolescents, accounting for approximately 0.5-2% of all neoplasms in these age groups. Annual incidence in children is approximately 0.5 cases per million. Because of the low incidence of this tumor type in children, performing a meaningful study of the many epidemiological factors beyond those listed below is difficult

·         Internationally: The international incidence is highly associated with endemic hepatitis B exposure. In China, aflatoxin exposure has been associated with the development of HCC in the fifth, sixth, and seventh decades of life.

Mortality/Morbidity: Morbidity and mortality directly correlate with surgical resectability of the primary tumor. Although chemotherapy and radiation control may improve in the clinical course, in selected patients, the overriding objective of these modalities is to render the tumor completely resectable. (See Surgical Care for details.)

Race: In older adults, race may play a role in the development of HCC, but it is difficult to exclude environmental factors from these determinations. Because the condition is so rare in adolescents and children (0.5 cases/million), ethnic data are not readily available for these age groups. Most studies of HCC have involved patients of Asian descent.

Sex: Because most congenital forms of liver dysfunction (eg, urea cycle defects, storage diseases, hereditary hemochromatosis) are inherited in an autosomal recessive manner, the female-to-male occurrence ratio in children and adolescents is equal.

Age: The patient is usually an older school-aged child or adolescent, often with no preexisting diagnosis of cirrhotic liver disease. In patients with underlying liver dysfunction, the likelihood of developing the condition increases with age.

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