Hepatoblastoma

July 23, 2025

This page was reviewed under our medical and editorial policy by by Janet Yoon, M.D., clinical professor, medical director of the Pediatric Musculoskeletal Tumor Program, Department of Pediatrics, City of Hope^® Cancer Center Duarte. 

Hepatoblastoma is a rare ​​type of liver cancer that usually affects children aged 5 and under. It accounts for only 50 to 70 new cases in the United States every year, or about 1% of all childhood cancer diagnoses.

What Is Hepatoblastoma?

Hepatoblastoma is a cancer that develops in the tissue of the liver. Almost all cases of hepatoblastoma occur in children, with the most common age of diagnosis being 3 years old.

The liver, which is located in the middle of the abdomen, is one of the body’s largest organs. It performs several vital functions, including filtering harmful substances out of the bloodstream, storing sugar that is used to provide the body with energy and producing bile, which helps the body digest fat.

A patient’s cancer care team usually classifies hepatoblastoma based on what the cancer cells look like under a microscope. The different classifications, which refer to whether the cells look more like fetal cells or more like mature cells, are:

• Well-differentiated fetal (or pure fetal) hepatoblastoma
• Small-cell undifferentiated hepatoblastoma
• Mixed epithelial and fetal hepatoblastoma

Hepatoblastoma Symptoms

Some of the common symptoms of hepatoblastoma include:

• A swelling or lump in the abdomen that grows quickly and is somewhat painful
• Higher-than-normal levels of a substance called alpha-fetoprotein (AFP), which is a protein produced in the liver
• Unexplained weight loss, though this is less common

What Causes Hepatoblastoma?

The exact cause of hepatoblastoma is not yet fully understood. But scientists and doctors are continuing to study what may prompt it to develop. There are some factors that may increase a person's risk for developing hepatoblastoma.

Risk Factors

A risk factor is something that makes it more likely than average that someone will develop a certain condition. Some risk factors for hepatoblastoma include:

• Having certain genetic conditions, such as Simpson-Golabi-Behmel syndrome, Aicardi syndrome or Beckwith-Wiedemann syndrome
• Genetic disorders like trisomy 18
• Being born prematurely and with a low weight
• Hemihyperplasia, a condition which makes one side of a child’s body larger than the other
• Having familial adenomatous polyposis, a condition in which abnormal small growths called polyps develop in the colon and intestines
• Glycogen storage disease, a condition in which the body cannot store and process a particular form of sugar properly

Diagnosis and Testing

Cancer doctors may recommend a range of tests to help diagnose hepatoblastoma. Usually, these tests take place after a physical examination and a discussion of family medical history.

Tumor marker testing: Certain kinds of tests look for unusually high or low levels of substances in the body that may be a sign of cancer. One common tumor marker blood test for hepatoblastoma is an ​alpha fetoprotein (AFP) test (which looks for elevated levels of the AFP protein).

Complete ​​blood count and ​liver function tests: These tests measure the levels of different kinds of blood cells, proteins and other substances in the body, which may help determine whether the liver is functioning normally.

Imaging tests: Ultrasound, magnetic resonance imaging or computed tomography may help detect tumors in the liver or look for changes to liver function.

Biopsy: This is a procedure in which a small tissue sample is removed from the body and examined under a microscope for signs of cancer.

Stages

When diagnosing cancer, doctors stage the disease, which means using certain criteria to determine how far the cancer has spread and how much cancer is in the body. The first step in staging hepatoblastoma is to categorize the disease into one of the following two groups.

PRETEXT (or pretreatment extent): This refers to the cancer’s stage before treatment.

POSTTEXT (or post-treatment extent): This refers to the cancer’s stage after treatment has begun.

When staging hepatoblastoma, doctors look at four different sections of the liver. They then stage the cancer based on where tumors are located in the liver and whether treatment has begun yet. The stages of hepatoblastoma are described as PRETEXT or POSTTEXT 1, 2, 3 or 4, as detailed below.

PRETEXT 1 or POSTTEXT 1: Cancer is in only one of the four sections of the liver.

PRETEXT 2 or POSTTEXT 2: Cancer is in one or two sections of the liver, and there are still two adjacent sections of the liver that do not have any cancer.

PRETEXT 3 or POSTTEXT 3: Cancer is in two sections of the liver that are not adjacent, or in three sections of the liver in total.

PRETEXT 4 or POSTTEXT 4: Cancer is in all four sections of the liver.

Hepatoblastoma Treatment

Cancer doctors may recommend one or several different treatment options for hepatoblastoma, depending on factors such as the cancer’s stage, how old the patient is and whether he or she is experiencing any other health issues.

Surgery

Surgery is the most common treatment for hepatoblastoma. Several different types of surgery are available, including removal of part of the liver (partial ​​hepatectomy), removal of all of the liver followed by a liver transplant and surgical removal of any cancer that has spread outside of the liver. Patients often receive other treatments alongside surgery, with the goal of making the procedure as effective as possible.

Chemotherapy

​Chemotherapy is the use of medications that are designed to kill cancer cells. Systemic chemotherapy is delivered into a vein and may affect healthy cells as well as cancer cells.

Radiation therapy

Several forms of ​​radiation therapy are used to treat hepatoblastoma. They all use radiation to target and destroy cancer cells. External radiation therapy involves aiming beams of radiation at a tumor from outside the body. Internal radiation therapy uses small radioactive seeds that are placed inside the body at or near the site of a tumor. Radioembolization is another option, which involves injecting a radioactive substance into the main artery delivering blood to the liver, with the goal of cutting off the blood supply to a tumor.

Who Treats Hepatoblastoma?

Children with hepatoblastoma are cared for by a multidisciplinary team of ​​pediatric cancer experts and clinical providers, including:

• Pediatric oncologists with expertise in treating ​types of childhood cancers
• Surgical oncologists, who are experts in complex surgical techniques for removing tumors from the body
• Gastroenterologists, who specialize in treating diseases of the gastrointestinal tract
• Radiation oncologists, who have expertise in innovative radiation therapy techniques
• Supportive care staff specializing in providing physical and emotional support to patients and their loved ones
• Trained oncology nurses, who care for patients during their hospital treatments and stays

Can Hepatoblastoma Come Back After Treatment?

It is possible for hepatoblastoma to recur, or come back, even after treatment. If a child is diagnosed with recurrent hepatoblastoma, his or her cancer care team will work closely with loved ones to determine the most effective treatment options.

Hepatoblastoma Survival Rate

The prognosis for children who are diagnosed with hepatoblastoma depends on factors such as how early cancer was detected and how far it has spread before treatment begins. The five-year survival rate for early-stage hepatoblastoma is approximately 90%. The overall five-year survival rate for all stages of the disease is between 60% and 70%.