Hepatocellular carcinoma in children is a disease in which malignant (cancerous) cells form in the liver tissue.
The liver is one of the largest organs in the body. It can be divided into four lobes and is located in the upper right side of the abdomen within the chest cavity. The three important functions of the liver are:
Filtering harmful substances from the blood and removing them from the body through feces and urine.
Secrete bile, which facilitates the body’s digestion of fats in food.
Storage of glycogen (sugar), which provides energy to the body. 
Anatomy of the liver. The liver is located in the upper abdomen near the stomach, intestine, gallbladder, and pancreas. The liver has a right lobe and a left lobe. Each lobe is divided into two parts (not shown).
Hepatocellular carcinoma is rare in children and adolescents.
There are different types of childhood liver cancer.
There are two main types of childhood liver cancer:
Hepatoblastoma: Hepatoblastoma is the most common type of childhood liver cancer. It usually affects children younger than 3 years of age.
In hepatoblastoma, the histology (how the cancer cells look under the microscope) affects how the cancer is treated. The histology of hepatoblastoma may be one of the following:
Highly differentiated fetal (pure fetal) histology
Small cell undifferentiated histology
Non-highly differentiated fetal histology, non-small cell undifferentiated histology
Hepatocellular carcinoma: Hepatocellular carcinoma usually affects older children and adolescents. It is more common in Asia, where there is a high prevalence of hepatitis B infection, compared with the United States.
Other less common types of childhood liver cancer include:
Undifferentiated embryonal sarcoma of the liver: This type of liver cancer usually occurs in children 5 to 10 years of age. It usually spreads throughout the liver and/or lungs.
Infant hepatic choriocarcinoma: This is a very rare tumor that begins in the placenta and spreads to the fetus. The tumor is usually found within the first few months of life. In addition, the mother of the child may be diagnosed with choriocarcinoma. Choriocarcinoma is a trophoblastic disease of pregnancy. For more information on the treatment of choriocarcinoma in mothers of children, see the PDQ summary on the treatment of gestational trophoblastic disease.
Vascular liver tumors: These tumors form in the liver and consist of cells that form blood vessels or lymphatic vessels. Vascular liver cancer can be benign (not cancerous) or malignant (cancerous). For more information about vascular liver tumors, see the PDQ summary on the treatment of vascular tumors in children.
This abstract is about the treatment of primary liver cancer (cancer that starts in the liver). Treatment of metastatic liver cancer, which is cancer that starts elsewhere in the body and spreads to the liver, is not discussed in this abstract.
Primary liver cancer can occur in both adults and children. However, treatment for children is different from treatment for adults. For more information about adult treatment, see the PDQ summary on the treatment of primary liver cancer in adults.
Some diseases and conditions can increase the risk of liver cancer in children.
Any factor that increases the chance of getting the disease is called a risk factor. Having a risk factor does not mean that you will definitely get cancer; not having a risk factor does not mean that you will not get cancer. If you think your child is at risk, consult your doctor.
Risk factors for hepatoblastoma include the following syndromes or conditions:
Echaldi syndrome
Burr-Wey syndrome
Hemifacial hypertrophic hyperplasia
Familial adenomatous polyposis (FAP)
Gluconeogenic storage disease
Very low birth weight
Degree growth syndrome
Some genetic changes, such as trisomy 18
Children at risk for hepatoblastoma can be tested for cancer before any symptoms appear. An abdominal ultrasound is performed every 3 months until the child is 4 years old, and blood levels of methemoglobin are checked.
Risk factors for hepatocellular carcinoma include the following syndromes or conditions:
Alagio syndrome
Gluconeogenic storage disease
Hepatitis B virus infection transmitted from mother to child at birth
Progressive familial intrahepatic disease
Tyrosinemia
Some patients with tyrosinemia will undergo liver transplantation before signs or symptoms of cancer develop.
Signs and symptoms of liver cancer in children include an abdominal mass or pain.
Patients will have signs and symptoms more often after the tumor has grown in size. Other diseases may also cause the same signs and symptoms. Contact your doctor if your child has any of the following symptoms:
A lump in the abdomen that may be painful.
Swelling in the abdomen
Unexplained weight loss.
Loss of appetite
Nausea and vomiting.
Tests used to examine the liver and blood can be used to identify (find) and diagnose liver cancer in children and to determine if the cancer has spread.
The following tests and procedures may be involved:
Physical examination and medical history: An examination of the body to check for general signs of health, including examination for signs of disease, such as lumps or anything that looks abnormal. The patient’s health habits, previous illnesses, and treatment history are also referred to.
Serum tumor marker test: A test that checks the concentration of secretions from organs, tissues, or tumor cells in the body in a blood sample. Elevated levels of certain substances in the blood are associated with specific types of cancer. These are called tumor markers. The blood of a child with liver cancer may contain the hormone called beta-human chorionic gonadotropin (beta-hCG) or the protein called alpha-fetoprotein (AFP). Other cancers, benign liver tumors, and certain noncancerous diseases, including cirrhosis and hepatitis, can also increase AFP levels.
Complete blood count (CBC): A method of taking a blood sample and checking for.
Red blood cell, white blood cell, and platelet counts.
The amount of hemoglobin (the protein that carries oxygen) in red blood cells.
The portion of the blood sample that is made up of red blood cells
Liver function test: a method of checking the amount of liver secreted substances in a blood sample. If it is higher than normal, there may be damage or cancer in the liver.
Blood chemistry verification: A method to check the amount of certain substances (such as bilirubin or lactate dehydrogenase (LDH)) released by organs and tissues in a blood sample. Abnormal levels of a substance (higher or lower than normal) may be a sign of disease.
Epstein-Barr virus (EBV) test: A blood test that checks for antibodies to EBV and markers of EBV DNA (which can be detected in the blood of patients with EBV infection) in blood samples.
Hepatitis test: A blood test to check for the presence of hepatitis virus in blood samples.
MRI (magnetic resonance imaging) using gadolinium: A method that uses magnets, radio waves, and a computer to image the inner areas of the liver in a series of details. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells, brightening the display in the light slices. This method is also known as nuclear magnetic resonance imaging (NMRI). 
Abdominal magnetic resonance imaging (MRI) Imaging (MRI). The child lies flat on a table, slides into the MRI scanner, and takes a light film of the inside of the body. A pad on the child’s abdomen makes the light film clearer.
CT scan (CAT scan): A method of taking a series of detailed images of areas inside the body from different angles by connecting a computer to an x-ray machine. Dye may need to be injected into a vein or swallowed to improve the clarity of the organ or tissue displayed, also called computed tomography, computed tomography, or computerized axial level radiography. In the diagnosis of liver cancer in children, CT scans of the chest and abdomen are usually required. 
Computed tomography (CT) scan of the abdomen. The child lies on a table and slides into a CT scanner that takes x-rays of the inside of the abdomen.
Ultrasound: An exam in which high-energy sound waves (ultrasound) are reflected from internal tissues or organs and produce echoes. These echoes form an image of the body’s tissues called a sonogram. Such images can be printed out for later examination. In children with liver cancer, ultrasound of the abdomen is often performed to check for large blood vessels. 
Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer reflects sound waves away from internal organs and tissues, producing echoes that form a sonogram (computer image).
Abdominal x-ray: An x-ray of the abdominal organs is performed. x-ray is an energy ray that penetrates the body and is displayed on film to produce images of the body.
Biopsy: A sample of cells or tissue is removed so that it can be looked at under a microscope to check for signs of cancer. The sample may be removed during surgery or to look at the tumor. The pathologist looks at the sample under the microscope to find out what type of liver cancer is present.
The following tests can be done on removed tissue samples:
Immunohistochemistry: A laboratory test that uses antibodies to check for certain antigens (markers) in a patient’s tissue sample. Antibodies are usually linked to enzymes or fluorescent dyes. After the antibody binds to a specific antigen in the tissue sample, the enzyme or dye is activated and the antigen can then be seen under a microscope. Such tests are used to check for a certain genetic mutation, to help diagnose cancer, and to help distinguish between different kinds of cancer.
Several factors can affect the prognosis (chance of recovery) and the choice of treatment options.
The prognosis (chance of recovery) and treatment options for hepatoblastoma depend on the following factors:
PRETEXT group
Size of the tumor
The type of hepatoblastoma whether it is highly differentiated fetal (pure fetal) or small cell undifferentiated histology
Whether the cancer has spread to other parts of the body, such as the diaphragm, lungs, or certain large blood vessels
Whether more than one tumor is present in the liver.
Whether the overlying layer around the tumor has ruptured.
How the cancer responds to chemotherapy
Whether the tumor can be completely removed by surgery
Whether the patient can have a liver transplant
Whether the methemoglobin blood level decreases after treatment
The age of the child
Whether the cancer has just been diagnosed or is a recurrence.
The prognosis (chance of recovery) and treatment options for hepatocellular carcinoma depend on the following factors:
PRETEXT group
Whether the cancer has spread to other parts of the body, such as the lungs.
Whether the tumor can be completely removed by surgery.
The cancer’s response to chemotherapy.
Whether the child has hepatitis B infection.
Whether the cancer has just been diagnosed or is a recurrence.
For children with liver cancer that has recurred (reoccurred) after initial treatment, the prognosis and choice of treatment options depends on:
The site of tumor recurrence.
Treatment measures at the time of initial presentation.
Hepatocellular carcinoma in children is curable if the tumor is small and can be completely removed by surgery. Hepatoblastoma is easier to completely resect than hepatocellular carcinoma.