Pediatric Hepatocellular Carcinoma Clinical Presentation

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

History

Elements to ascertain include a prior history of hepatitis B or hepatitis C, chronic cirrhosis, or other diseases that tend to induce liver dysfunction. Co-infection with human immunodeficiency virus (HIV) may further enhance a patient's risk for developing hepatocellular carcinoma (HCC). Most patients complain of abdominal pain, weight loss, and diminished appetite. In patients with a history of chronic liver disease, a change in routine symptoms may indicate the presence of a liver tumor.

Most patients with hepatocellular carcinoma present with a slowly enlarging, right upper-quadrant mass that may be found during a routine physical examination, brought to medical attention by the patient, or discovered by the patient's parents. Many children also experience localized pain, nausea, vomiting, and weight loss. Nearly 25% of patients present with jaundice.

In adults, chronic hepatitis secondary to alcohol exposure, infection with hepatitis, and hereditary hemochromatosis are predisposing factors. Aflatoxins and other environmental factors also are likely to play a role in the pathogenesis in adults. In comparison, children are far more likely to have inherited errors of metabolism, such as tyrosinemia or urea cycle enzymopathies. Liver diseases that cause cirrhosis increase risk for developing hepatocellular carcinoma (eg, alpha-1 antitrypsin deficiency).

Children with biliary atresia, chronic cholestasis, or glycogen-storage diseases are at increased risk. Symptoms can be masked in children with preexisting hepatic diseases, and, accordingly, a change in a chronic disease pattern merits careful consideration for the possibility of a new malignancy.

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Physical

The physical examination often reveals abnormalities attributable to a hepatic tumor. In advanced cases, or when the primary tumor is large, the liver may be palpable below the right costal margin. In addition, deep palpation often reveals pain, especially over the location of the liver. Scleral icterus and other signs of jaundice are frequent. The patient's history also may indicate weight loss, the extent of which may be observed during the examination. In patients in whom metastatic disease to the lungs is in question, percussion of the lungs may reveal a difference in density, suggesting a pleural effusion. Other painful sites discovered on the examination should lead to radiographic imaging to determine the extent of malignant spread. This is particularly true for bone pain at presentation.

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Causes

Although no cause has been clearly elucidated, the risk factors for children and adolescents include a history of hepatitis B or C, alpha-1 antitrypsin deficiency, hereditary tyrosinemia, Gaucher disease, congenital biliary atresia, urea cycle defects, severe iron overload (as occurs with thalassemia or sickle cell disease requiring chronic blood transfusion), or other forms of chronic cirrhosis or liver dysfunction. Acquired hepatitis C from blood product transfusions is an important risk factor because the risk of hepatocellular carcinoma in patients with chronic hepatitis C and cirrhosis is highest (2-8% per year).

In areas of the world where hepatitis B or C are endemic, the incidence is likely to be proportionally increased in children and adolescents.

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