Signs and symptoms of hepatoblastoma

Signs and symptoms are more common after the tumor gets big. Other conditions can cause the same signs and symptoms. Check with your child’s doctor if your child has any of the following:

• a lump in the abdomen that may be painful
• swelling in the abdomen
• weight loss for no known reason
• loss of appetite
• nausea and vomiting

Causes and risk factors for hepatoblastoma

Anything that increases your chance of getting a disease is called a risk factor. Not every child with one or more of these risk factors will develop hepatoblastoma, and hepatoblastoma will develop in some children who don’t have any known risk factors. Talk with your child’s doctor if you think your child may be at risk.

The following syndromes or conditions are risk factors for hepatoblastoma:

• Aicardi syndrome
• Beckwith-Wiedemann syndrome
• hemihyperplasia
• familial adenomatous polyposis (FAP)
• glycogen storage disease
• a very low weight at birth
• Simpson-Golabi-Behmel syndrome
• certain genetic changes, such as trisomy 18

Children at risk of developing hepatoblastoma may have tests done to check for cancer before any symptoms appear. Every 3 months until the child is 4 years old, an abdominal ultrasound exam is done and the level of alpha-fetoprotein in the blood is checked.

Diagnosis of hepatoblastoma

Tests that examine the liver and the blood are used to diagnose hepatoblastoma and find out whether the cancer has spread.

The following tests and procedures may be used:

• Physical exam and health history: A physical exam of the body will be done to check a person’s health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
• Serum tumor marker test: This blood test measures the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The blood of children who have liver cancer may have increased amounts of a hormone called beta-human chorionic gonadotropin (beta-hCG) or a protein called alpha-fetoprotein (AFP). Other cancers, benign liver tumors, and certain noncancer conditions, including cirrhosis and hepatitis, can also increase AFP levels.
• Complete blood count (CBC): A sample of blood is drawn and checked for the following:

  -

  the number of red blood cells, white blood cells, and platelets

  -

  the amount of hemoglobin (the protein that carries oxygen) in the red blood cells

  -

  the portion of the blood sample made up of red blood cells

• Liver function tests: These blood tests measure the amounts of certain substances released into the blood by the liver. A higher-than-normal amount of a substance can be a sign of liver damage or cancer.
• Blood chemistry studies: These blood tests measure the amounts of certain substances, such as bilirubin or lactate dehydrogenase (LDH), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
• Epstein-Barr virus (EBV) test: This blood test checks for antibodies to the EBV and DNA markers of the EBV. These are found in the blood of patients who have been infected with EBV.
• Magnetic resonance imaging (MRI) with gadolinium: This procedure uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the liver. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture.

  

  Magnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.

• CT scan (CAT scan): This procedure uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, taken from different angles. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. In childhood liver cancer, a CT scan of the chest and abdomen is usually done.

  

  Computed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.

• Ultrasound exam: This procedure uses high-energy sound waves (ultrasound) that are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. In childhood liver cancer, an ultrasound exam of the abdomen to check the large blood vessels is usually done.

  

  Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).

• Abdominal x-ray: An x-ray of the organs in the abdomen may be done. An x-ray is a type of energy beam that can go through the body onto film, making a picture of areas inside the body.
• Biopsy: During a biopsy, a doctor removes a sample of cells or tissue. A pathologist then views the cells or tissue under a microscope to look for cancer cells and find out the type of cancer. The doctor may remove as much tumor as safely possible during the same biopsy procedure.
  The following test may be done on the sample of tissue that is removed:

  • Immunohistochemistry: This laboratory test uses antibodies to check for certain antigens (markers) in a sample of a patient’s tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to check for a certain gene mutation, to help diagnose cancer, and to help tell one type of cancer from another type of cancer.

Prognostic factors for hepatoblastoma

The prognosis (chance of recovery) and treatment options for hepatoblastoma depend on the following:

• the PRETEXT group
• the size of the tumor
• whether the type of hepatoblastoma is well-differentiated fetal (pure fetal) or small cell undifferentiated histology
• whether the cancer has spread to other places in the body, such as the diaphragm, lungs, or certain large blood vessels
• whether there is more than one tumor in the liver
• whether the outer covering around the tumor has broken open
• how the cancer responds to chemotherapy
• whether the cancer can be removed completely by surgery
• whether the patient can have a liver transplant
• whether the AFP blood levels go down after treatment
• the age of the child
• whether the cancer has just been diagnosed or has recurred

For hepatoblastoma that recurs (comes back) after initial treatment, the prognosis and treatment options depend on the following:

• where in the body the tumor recurred
• the type of treatment used to treat the initial cancer

Hepatoblastoma may be cured if the tumor is small and can be completely removed by surgery.

Stages of hepatoblastoma

After hepatoblastoma has been diagnosed, tests are done to find out if cancer cells have spread within the liver or to other parts of the body. The process used to find out if cancer has spread within the liver, to nearby tissues or organs, or to other parts of the body is called staging. In hepatoblastoma, the PRETEXT and POSTTEXT groups are used instead of stage to plan treatment. The results of the tests and procedures done to detect, diagnose, and find out whether the cancer has spread are used to determine the PRETEXT and POSTTEXT groups.

Two grouping systems are used for hepatoblastoma to decide whether the tumor can be removed by surgery:

• The PRETEXT group describes the tumor before the patient has any treatment.
• The POSTTEXT group describes the tumor after the patient has had treatment such as neoadjuvant chemotherapy.

The liver is divided into four sections. The PRETEXT and POSTTEXT groups depend on which sections of the liver have cancer. There are four PRETEXT and POSTTEXT groups.

PRETEXT and POSTTEXT group I

Liver PRETEXT and POSTTEXT I. Cancer is found in one section of the liver. Three sections of the liver that are next to each other do not have cancer in them.

In group I, the cancer is found in one section of the liver. Three sections of the liver that are next to each other do not have cancer in them.

PRETEXT and POSTTEXT group II

Liver PRETEXT and POSTTEXT II. Cancer is found in one or two sections of the liver. Two sections of the liver that are next to each other do not have cancer in them.

In group II, cancer is found in one or two sections of the liver. Two sections of the liver that are next to each other do not have cancer in them.

PRETEXT and POSTTEXT group III

Liver PRETEXT and POSTTEXT III. Cancer is found in three sections of the liver and one section does not have cancer in it, or cancer is found in two sections of the liver and two sections that are not next to each other do not have cancer in them.

In group III, one of the following is true:

• Cancer is found in three sections of the liver and one section does not have cancer.
• Cancer is found in two sections of the liver and two sections that are not next to each other do not have cancer in them.

PRETEXT and POSTTEXT group IV

Liver PRETEXT and POSTTEXT IV. Cancer is found in all four sections of the liver.

In group IV, cancer is found in all four sections of the liver.

Sometimes hepatoblastoma continues to grow or comes back after treatment.

Progressive disease is cancer that continues to grow, spread, or worsen. Progressive disease may be a sign that the cancer has become refractory to treatment.

Recurrent hepatoblastoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the liver or in other parts of the body. To learn more about metastatic cancer (cancer that has spread from where it started to other parts of the body), see Metastatic Cancer: When Cancer Spreads.

Types of treatment for hepatoblastoma

Children with hepatoblastoma should have their treatment planned by a team of healthcare providers who are experts in treating this rare childhood cancer. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other healthcare providers who are experts in treating children with hepatoblastoma and who specialize in certain areas of medicine. It is especially important to have a pediatric surgeon with experience in liver surgery who can send patients to a liver transplant program if needed. Other specialists may include the following:

• pediatrician
• radiation oncologist
• pediatric nurse specialist
• rehabilitation specialist
• psychologist
• social worker
• nutritionist

For more information about having a child with cancer and ways to cope and find support, see Childhood Cancers.

Every child will not receive all the treatments listed below. Your child’s care team will help you make treatment decisions based on your child’s unique situation. To learn more about factors that help determine the treatment plan for hepatoblastoma, see Prognostic factors for hepatoblastoma.

Treatment of newly diagnosed hepatoblastoma

Treatment options for newly diagnosed hepatoblastoma that can be removed by surgery at the time of diagnosis may include the following:

• surgery to remove the tumor, followed by combination chemotherapy for hepatoblastoma that is not well-differentiated fetal histology or aggressive chemotherapy for small cell undifferentiated histology
• surgery to remove the tumor, followed by watchful waiting or chemotherapy, for hepatoblastoma with well-differentiated fetal histology

Treatment options for newly diagnosed hepatoblastoma that cannot be removed by surgery or is not removed at the time of diagnosis may include the following:

• combination chemotherapy to shrink the tumor, followed by surgery to remove the tumor
• combination chemotherapy, followed by a liver transplant
• chemoembolization of the hepatic artery to shrink the tumor, followed by surgery to remove the tumor
• if the tumor in the liver cannot be removed by surgery but there are no signs of cancer in other parts of the body, the treatment may be a liver transplant

For newly diagnosed hepatoblastoma that has spread to other parts of the body at the time of diagnosis, combination chemotherapy is given to shrink the tumors in the liver and cancer that has spread to other parts of the body. After chemotherapy, imaging tests are done to check whether the tumors can be removed by surgery.

Treatment options may include the following:

• If the tumor in the liver and other parts of the body (usually nodules in the lung) can be removed, surgery will be done to remove the tumors followed by chemotherapy to kill any cancer cells that may remain.
• If the tumor in other parts of the body cannot be removed or a liver transplant is not possible, chemotherapy, chemoembolization of the hepatic artery, or radiation therapy may be given.
• If the tumor in other parts of the body cannot be removed or the patient does not want surgery, radiofrequency ablation may be given.

Treatment options in clinical trials for newly diagnosed hepatoblastoma include the following:

• a clinical trial of chemotherapy and surgery

Treatment of progressive or recurrent hepatoblastoma

Treatment of progressive or recurrent hepatoblastoma may include the following:

• surgery to remove isolated (single and separate) metastatic tumors with or without chemotherapy
• radiofrequency ablation
• combination chemotherapy
• liver transplant
• ablation therapy (radiofrequency ablation or percutaneous ethanol injection) as palliative therapy to relieve symptoms and improve the quality of life
• a clinical trial that checks a sample of the patient's tumor for certain gene changes to determine the type of targeted therapy that will be given to the patient

Signs and symptoms of childhood hepatocellular carcinoma 

Signs and symptoms are more common after the tumor gets big. Other conditions can cause the same signs and symptoms. Check with your child’s doctor if your child has any of the following: 

• a lump in the abdomen that may be painful 
• swelling in the abdomen 
• weight loss for no known reason 
• loss of appetite 
• nausea and vomiting 

Causes and risk factors for childhood hepatocellular carcinoma

Anything that increases your chance of getting a disease is called a risk factor. Not every child with one or more of these risk factors will develop hepatocellular carcinoma, and hepatocellular carcinoma will develop in some children who don’t have any known risk factors. Talk with your child’s doctor if you think your child may be at risk.  

The following syndromes or conditions are risk factors for childhood hepatocellular carcinoma: 

• Alagille syndrome. 
• Glycogen storage disease. 
• Hepatitis B virus infection that was passed from mother to child at birth.
• Progressive familial intrahepatic disease. 
• Tyrosinemia: Some patients with tyrosinemia are diagnosed with hepatocellular carcinoma after receiving a liver transplant, before there are signs or symptoms of cancer.

Hepatocellular carcinoma may develop in children with no underlying liver disease.

Diagnosis of childhood hepatocellular carcinoma

Tests that examine the liver and the blood are used to diagnose childhood hepatocellular carcinoma and find out whether it has spread. 

The following tests and procedures may be used: 

• Physical exam and health history: A physical exam of the body will be done to check a person’s health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. 
• Serum tumor marker test: This blood test measures the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The blood of children who have liver cancer may have increased amounts of a hormone called beta-human chorionic gonadotropin (beta-hCG) or a protein called alpha-fetoprotein (AFP). Other cancers, benign liver tumors, and certain noncancer conditions, including cirrhosis and hepatitis, can also increase AFP levels. 
• Complete blood count (CBC): A sample of blood is drawn and checked for the following:

  -

  the number of red blood cells, white blood cells, and platelets 

  -

  the amount of hemoglobin (the protein that carries oxygen) in the red blood cells

  -

  the portion of the blood sample made up of red blood cells 

• Liver function tests: These blood tests measure the amounts of certain substances released into the blood by the liver. A higher-than-normal amount of a substance can be a sign of liver damage or cancer. 
• Blood chemistry studies: These blood tests measure the amounts of certain substances, such as bilirubin or lactate dehydrogenase (LDH), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.   
• Epstein-Barr virus (EBV) test: This blood test checks for antibodies to the EBV and DNA markers of the EBV. These are found in the blood of patients who have been infected with EBV. 
• Hepatitis assay: This blood test checks for pieces of the hepatitis virus. 
• Magnetic resonance imaging (MRI) with gadolinium: This procedure uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the liver. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. 

  

  Magnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.

• CT scan (CAT scan): This procedure uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, taken from different angles. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. In childhood liver cancer, a CT scan of the chest and abdomen is usually done.  

  

  Computed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.

• Ultrasound exam: This procedure uses high-energy sound waves (ultrasound) that are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. In childhood liver cancer, an ultrasound exam of the abdomen to check the large blood vessels is usually done. 

  

  Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).

• Abdominal x-ray: An x-ray of the organs in the abdomen may be done. An x-ray is a type of energy beam that can go through the body onto film, making a picture of areas inside the body. 
• Biopsy: During a biopsy, a doctor removes a sample of cells or tissue. A pathologist then views the cells or tissue under a microscope to look for cancer cells and find out the type of cancer. The doctor may remove as much tumor as safely possible during the same biopsy procedure.
  The following test may be done on the sample of tissue that is removed: 

  • Immunohistochemistry: This laboratory test uses antibodies to check for certain antigens (markers) in a sample of a patient’s tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to check for a certain gene mutation, to help diagnose cancer, and to help tell one type of cancer from another type of cancer. 

Prognostic factors for childhood hepatocellular carcinoma

The prognosis (chance of recovery) and treatment options for childhood hepatocellular carcinoma depend on the following:

• the PRETEXT group 
• whether the cancer has spread to other places in the body, such as the lungs 
• whether the cancer can be removed completely by surgery 
• how the cancer responds to chemotherapy 
• whether the child has hepatitis B virus infection 
• whether the cancer has just been diagnosed or has recurred 

For childhood hepatocellular carcinoma that recurs (comes back) after initial treatment, the prognosis and treatment options depend on the following:

• where in the body the tumor recurred 
• the type of treatment used to treat the initial cancer 

Childhood liver cancer may be cured if the tumor is small and can be completely removed by surgery. Complete removal is possible more often for hepatoblastoma than for hepatocellular carcinoma. 

Stages of childhood hepatocellular carcinoma  

After childhood hepatocellular carcinoma has been diagnosed, tests are done to find out if cancer cells have spread within the liver or to other parts of the body. The process used to find out if cancer has spread within the liver, to nearby tissues or organs, or to other parts of the body is called staging. In childhood hepatocellular carcinoma, the PRETEXT and POSTTEXT groups are used instead of stage to plan treatment. The results of the tests and procedures done to detect, diagnose, and find out whether the cancer has spread are used to determine the PRETEXT and POSTTEXT groups. 

Two grouping systems are used for childhood hepatocellular carcinoma to decide whether the tumor can be removed by surgery:

• The PRETEXT group describes the tumor before the patient has any treatment. 
• The POSTTEXT group describes the tumor after the patient has had treatment such as neoadjuvant chemotherapy. 

The liver is divided into four sections. The PRETEXT and POSTTEXT groups depend on which sections of the liver have cancer. 

PRETEXT and POSTTEXT group I  

Liver PRETEXT and POSTTEXT I. Cancer is found in one section of the liver. Three sections of the liver that are next to each other do not have cancer in them.

In group I, the cancer is found in one section of the liver. Three sections of the liver that are next to each other do not have cancer in them. 

PRETEXT and POSTTEXT group II  

Liver PRETEXT and POSTTEXT II. Cancer is found in one or two sections of the liver. Two sections of the liver that are next to each other do not have cancer in them.

In group II, cancer is found in one or two sections of the liver. Two sections of the liver that are next to each other do not have cancer in them. 

PRETEXT and POSTTEXT group III 

Liver PRETEXT and POSTTEXT III. Cancer is found in three sections of the liver and one section does not have cancer in it, or cancer is found in two sections of the liver and two sections that are not next to each other do not have cancer in them.

In group III, one of the following is true: 

• Cancer is found in three sections of the liver and one section does not have cancer. 
• Cancer is found in two sections of the liver and two sections that are not next to each other do not have cancer in them. 

PRETEXT and POSTTEXT group IV

Liver PRETEXT and POSTTEXT IV. Cancer is found in all four sections of the liver.

In group IV, cancer is found in all four sections of the liver. 

Sometimes childhood hepatocellular carcinoma continues to grow or comes back after treatment. 

Progressive disease is cancer that continues to grow, spread, or worsen. Progressive disease may be a sign that the cancer has become refractory to treatment.  

Recurrent childhood hepatocellular carcinoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the liver or in other parts of the body.  To learn more about metastatic cancer (cancer that has spread from where it started to other parts of the body), see Metastatic Cancer: When Cancer Spreads. 

Types of treatment for childhood hepatocellular carcinoma 

Children with hepatocellular carcinoma should have their treatment planned by a team of healthcare providers who are experts in treating this rare childhood cancer. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other healthcare providers who are experts in treating children with hepatocellular carcinoma and who specialize in certain areas of medicine. It is especially important to have a pediatric surgeon with experience in liver surgery who can send patients to a liver transplant program if needed. Other specialists may include the following:

• pediatrician 
• radiation oncologist 
• pediatric nurse specialist 
• rehabilitation specialist 
• psychologist 
• social worker 
• nutritionist 

For more information about having a child with cancer and ways to cope and find support, see Childhood Cancers.

Every child will not receive all the treatments listed below. Your child’s care team will help you make treatment decisions based on your child’s unique situation. To learn more about factors that help determine the treatment plan for childhood hepatocellular carcinoma, see Prognostic factors for childhood hepatocellular carcinoma. 

Treatment of newly diagnosed childhood hepatocellular carcinoma 

Treatment options for newly diagnosed hepatocellular carcinoma that can be removed by surgery at the time of diagnosis may include the following:

• surgery alone to remove the tumor
• surgery to remove the tumor, followed by chemotherapy with cisplatin and doxorubicin
• combination chemotherapy, followed by surgery to remove the tumor

Treatment options for newly diagnosed hepatocellular carcinoma that cannot be removed by surgery and has not spread to other parts of the body at the time of diagnosis may include the following:

• chemotherapy to shrink the tumor, followed by surgery to completely remove the tumor 
• chemotherapy to shrink the tumor 

If surgery to completely remove the tumor is not possible, further treatment may include the following:

• liver transplant 
• chemoembolization of the hepatic artery to shrink the tumor, followed by surgery to remove the tumor or liver transplant 
• chemoembolization of the hepatic artery alone 
• chemoembolization followed by liver transplant 
• radioembolization of the hepatic artery as palliative therapy to relieve symptoms and improve the quality of life 

Treatment for newly diagnosed hepatocellular carcinoma that has spread to other parts of the body at the time of diagnosis may include the following:

• Combination chemotherapy to shrink the tumor, followed by surgery to remove as much of the tumor as possible from the liver and other places where cancer has spread. Studies have not shown that this treatment works well but some patients may have some benefit. 

Treatment options for newly diagnosed hepatocellular carcinoma related to hepatitis B virus (HBV) infection may include the following:

• surgery to remove the tumor
• antiviral drugs that treat infection caused by the hepatitis B virus

Treatment options in clinical trials for newly diagnosed hepatocellular carcinoma may include the following:

• a clinical trial of chemotherapy and surgery  

Treatment of progressive or recurrent childhood hepatocellular carcinoma

Treatment of progressive or recurrent hepatocellular carcinoma may include the following:

• chemoembolization of the hepatic artery to shrink the tumor before liver transplant 
• liver transplant 
• a clinical trial of targeted therapy (sorafenib or pazopanib)
• a clinical trial that checks a sample of the patient's tumor for certain gene changes to determine the type of targeted therapy that will be given 

Signs and symptoms of childhood undifferentiated embryonal sarcoma of the liver

Signs and symptoms are more common after the tumor gets big. These and other signs and symptoms may be caused by undifferentiated embryonal sarcoma of the liver or by other conditions. Check with your child’s doctor if your child has any of the following:

• a lump in the abdomen that may be painful
• swelling in the abdomen

Diagnosis of childhood undifferentiated embryonal sarcoma of the liver

Tests that examine the liver and the blood are used to diagnose childhood undifferentiated embryonal sarcoma of the liver and find out whether the cancer has spread.

The following tests and procedures may be used:

• Physical exam and health history: A physical exam of the body will be done to check a person’s health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
• Complete blood count (CBC): A sample of blood is drawn and checked for the following:

  -

  the number of red blood cells, white blood cells, and platelets

  -

  the amount of hemoglobin (the protein that carries oxygen) in the red blood cells

  -

  the portion of the blood sample made up of red blood cells

• Liver function tests: These blood tests measure the amounts of certain substances released into the blood by the liver. A higher-than-normal amount of a substance can be a sign of liver damage or cancer.
• Blood chemistry studies: These blood tests measure the amounts of certain substances, such as bilirubin or lactate dehydrogenase (LDH), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
• Magnetic resonance imaging (MRI) with gadolinium: This procedure uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the liver. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture.

  

  Magnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.

• CT scan (CAT scan): This procedure uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, taken from different angles. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. In childhood liver cancer, a CT scan of the chest and abdomen is usually done.

  

  Computed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.

• Ultrasound exam: This procedure uses high-energy sound waves (ultrasound) that are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. In childhood liver cancer, an ultrasound exam of the abdomen to check the large blood vessels is usually done.

  

  Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).

• Abdominal x-ray: An x-ray of the organs in the abdomen may be done. An x-ray is a type of energy beam that can go through the body onto film, making a picture of areas inside the body.
• Biopsy: During a biopsy, a doctor removes a sample of cells or tissue. A pathologist then views the cells or tissue under a microscope to look for cancer cells and find out the type of cancer. The doctor may remove as much tumor as safely possible during the same biopsy procedure.
  The following test may be done on the sample of tissue that is removed:

  -

  Immunohistochemistry: This laboratory test uses antibodies to check for certain antigens (markers) in a sample of a patient’s tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to check for a certain gene mutation, to help diagnose cancer, and to help tell one type of cancer from another type of cancer.

Prognostic factors for childhood undifferentiated embryonal sarcoma of the liver

The prognosis (chance of recovery) and treatment options depend on the following:

• the size of the tumor
• the health of the patient
• how the cancer responds to chemotherapy
• whether the cancer can be removed completely by surgery
• whether the patient can have a liver transplant
• whether the cancer has just been diagnosed or has recurred

For childhood undifferentiated embryonal sarcoma of the liver that recurs (comes back) after initial treatment, the prognosis and treatment options depend on the following:

• where in the body the tumor recurred
• the type of treatment used to treat the initial cancer

Types of treatment for childhood undifferentiated embryonal sarcoma of the liver

Children with undifferentiated embryonal sarcoma of the liver should have their treatment planned by a team of healthcare providers who are experts in treating this rare childhood cancer. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other healthcare providers who are experts in treating children with undifferentiated embryonal sarcoma of the liver and who specialize in certain areas of medicine. It is especially important to have a pediatric surgeon with experience in liver surgery who can send patients to a liver transplant program if needed. Other specialists may include the following:

• pediatrician
• radiation oncologist
• pediatric nurse specialist
• rehabilitation specialist
• psychologist
• social worker
• nutritionist

For more information about having a child with cancer and ways to cope and find support, see Childhood Cancers.

Every child will not receive all the treatments listed below. Your child’s care team will help you make treatment decisions based on your child’s unique situation. To learn more about factors that help determine the treatment plan for this cancer, see Prognostic factors for childhood undifferentiated embryonal sarcoma of the liver.

Treatment of newly diagnosed childhood undifferentiated embryonal sarcoma of the liver

Treatment of newly diagnosed childhood undifferentiated embryonal sarcoma of the liver may include the following:

• Combination chemotherapy may be used to shrink the tumor, followed by surgery to remove as much of the tumor as possible. Chemotherapy may also be given after surgery to remove the tumor.
• Surgery may be done to remove the tumor, followed by chemotherapy. A second surgery may be done to remove tumor that remains, followed by more chemotherapy.
• Liver transplant may be done if surgery to remove the tumor is not possible.

Treatment of progressive or recurrent childhood undifferentiated embryonal sarcoma of the liver

Sometimes childhood undifferentiated embryonal sarcoma of the liver continues to grow or comes back after treatment.

• Progressive disease is cancer that continues to grow, spread, or worsen. Progressive disease may be a sign that the cancer has become refractory to treatment.
• Recurrent childhood undifferentiated embryonal sarcoma of the liver is cancer that has recurred (come back) after it has been treated. The cancer may come back in the liver or in other parts of the body.

To learn more about metastatic cancer (cancer that has spread from where it started to other parts of the body), see Metastatic Cancer: When Cancer Spreads.

Treatment of recurrent childhood undifferentiated embryonal sarcoma of the liver may include the following:

• a clinical trial that checks a sample of the patient's tumor for certain gene changes to determine the type of targeted therapy that will be given

Signs and symptoms of infantile choriocarcinoma of the liver

Signs and symptoms are more common after the tumor gets big. These and other signs and symptoms may be caused by infantile choriocarcinoma or by other conditions. Check with your child’s doctor if your child has any of the following:

• a lump in the abdomen
• swelling in the abdomen
• hemorrhage

Diagnosis of infantile choriocarcinoma of the liver

Tests that examine the liver and the blood are used to diagnose infantile choriocarcinoma of the liver and find out whether the cancer has spread. The following tests and procedures may be used:

• Physical exam and health history: A physical exam of the body will be done to check a person’s health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
• Serum tumor marker test: This blood test measures the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. The blood of children who have liver cancer may have increased amounts of a hormone called beta-human chorionic gonadotropin (beta-hCG) or a protein called alpha-fetoprotein (AFP). Other cancers, benign liver tumors, and certain noncancer conditions, including cirrhosis and hepatitis, can also increase AFP levels.
• Complete blood count (CBC): A sample of blood is drawn and checked for the following:

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  the number of red blood cells, white blood cells, and platelets

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  the amount of hemoglobin (the protein that carries oxygen) in the red blood cells

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  the portion of the blood sample made up of red blood cells

• Liver function tests: These blood tests measure the amounts of certain substances released into the blood by the liver. A higher-than-normal amount of a substance can be a sign of liver damage or cancer.
• Blood chemistry studies: These blood tests measure the amounts of certain substances, such as bilirubin or lactate dehydrogenase (LDH), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
• Magnetic resonance imaging (MRI) with gadolinium: This procedure uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the liver. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture.

  

  Magnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged.

• CT scan (CAT scan): This procedure uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, taken from different angles. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. In childhood liver cancer, a CT scan of the chest and abdomen is usually done.

  

  Computed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body.

• Ultrasound exam: This procedure uses high-energy sound waves (ultrasound) that are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. In childhood liver cancer, an ultrasound exam of the abdomen to check the large blood vessels is usually done.

  

  Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).

• Abdominal x-ray: An x-ray of the organs in the abdomen may be done. An x-ray is a type of energy beam that can go through the body onto film, making a picture of areas inside the body.
• Biopsy: During a biopsy, a doctor removes a sample of cells or tissue. A pathologist then views the cells or tissue under a microscope to look for cancer cells and find out the type of cancer. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same biopsy procedure.
  The following test may be done on the sample of tissue that is removed:

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  Immunohistochemistry: This laboratory test uses antibodies to check for certain antigens (markers) in a sample of a patient’s tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to check for a certain gene mutation, to help diagnose cancer, and to help tell one type of cancer from another type of cancer.

Prognostic factors for infantile choriocarcinoma of the liver

The prognosis (chance of recovery) and treatment options for infantile choriocarcinoma of the liver depend on the following:

• the size of the tumor
• the health of the patient
• how the cancer responds to chemotherapy
• whether the cancer can be removed completely by surgery
• whether the patient can have a liver transplant
• whether the cancer has just been diagnosed or has recurred

For infantile choriocarcinoma of the liver that recurs (comes back) after initial treatment, the prognosis and treatment options depend on the following:

• where in the body the tumor recurred
• the type of treatment used to treat the initial cancer

Types of treatment for infantile choriocarcinoma of the liver

Children with infantile choriocarcinoma of the liver should have their treatment planned by a team of healthcare providers who are experts in treating this rare childhood cancer. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other healthcare providers who are experts in treating children with infantile choriocarcinoma of the liver and who specialize in certain areas of medicine. It is especially important to have a pediatric surgeon with experience in liver surgery who can send patients to a liver transplant program if needed. Other specialists may include the following:

• pediatrician
• radiation oncologist
• pediatric nurse specialist
• rehabilitation specialist
• psychologist
• social worker
• nutritionist

For more information about having a child with cancer and ways to cope and find support, see Childhood Cancers.

Every child will not receive all the treatments listed below. Your child’s care team will help you make treatment decisions based on your child’s unique situation. To learn more about factors that help determine the treatment plan for this cancer, see Prognostic factors for infantile choriocarcinoma of the liver.

Treatment of newly diagnosed infantile choriocarcinoma of the liver

Treatment of newly diagnosed infantile choriocarcinoma of the liver may include the following:

• surgery to remove the tumor
• combination chemotherapy to shrink the tumor, followed by surgery to remove the tumor
• chemotherapy to treat the tumor followed by liver transplant

Treatment of progressive or recurrent infantile choriocarcinoma of the liver

Sometimes infantile choriocarcinoma of the liver continues to grow or comes back after treatment.

• Progressive disease is cancer that continues to grow, spread, or worsen. Progressive disease may be a sign that the cancer has become refractory to treatment.
• Recurrent infantile choriocarcinoma of the liver is cancer that has recurred (come back) after it has been treated. The cancer may come back in the liver or in other parts of the body.

To learn more about metastatic cancer (cancer that has spread from where it started to other parts of the body), see Metastatic Cancer: When Cancer Spreads.

Treatment of progressive or recurrent infantile choriocarcinoma of the liver may include the following:

• a clinical trial that checks a sample of the patient's tumor for certain gene changes to determine the type of targeted therapy that will be given