Pediatric Hepatocellular Carcinoma

Updated: Nov 05, 2015
  • Author: Paulette Mehta, MD, MPH; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Overview

Background

Hepatocellular carcinoma (HCC) is an aggressive hepatic neoplasm that most commonly affects 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 hepatocellular carcinoma.

The 2 pathological subtypes are classic hepatocellular carcinoma 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 hepatocellular carcinoma follow.

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Pathophysiology

The pathophysiology of hepatocellular carcinoma is not clearly understood; however, underlying liver dysfunction, especially cirrhosis, is a predisposing condition. In contrast to adults, most pediatric hepatocellular carcinomas arise de-novo, without underlying liver abnormalities. [1] Karyotypic abnormalities are not common. Although children and adolescents are unlikely to have chronic liver disease, congenital liver disorders increase the chance of developing hepatocellular carcinoma. These findings are suggestive of a multihit model of malignant transformation in hepatic tissue.

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Epidemiology

Frequency

United States

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. This is the second most common hepatic malignancy in children after hepatoblastoma.

International

The international incidence is highly associated with endemic hepatitis B exposure such as Southeast Asia and sub-Saharan Africa. In China, aflatoxin exposure has been associated with the development of hepatocellular carcinoma 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 hepatocellular carcinoma; however, excluding environmental factors from these determinations is difficult. Because the condition is so rare in adolescents and children (0.5 cases per million population), ethnic data are not readily available for these age groups. Most studies of hepatocellular carcinoma 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. After congenital Hepatitis B infection the life time risk of developing hepatocellular carcinoma is 50% for men and 20% for women.

Age

The incidence is lower in infants and higher in children and adolescents. 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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