Hepatoblastoma - Meaning, Radiology, Life Expectancy, Risks

Liver cancer begins when the cells in the liver start multiplying and dividing (proliferating) uncontrollably (abnormally). Liver cancers are classified into two categories, primary liver cancer and secondary liver cancer. Primary cancer occurs within the liver cells, whereas secondary cancer starts in some other body part and eventually spreads into the liver. 

Hepatoblastoma is one such primary cancer. It is a rare liver cancer that originates from the liver and accounts for around 1% of all childhood or paediatric cancers. In most instances, hepatoblastoma is seen in children under five years and is usually first seen during the first 18 months of life.  

This article highlights hepatoblastoma, its stages, signs and symptoms, causes, and risk factors. Please keep reading to learn more about everything you need to know about hepatoblastoma.

Hepatoblastoma is a primary liver cancer seen most commonly in children. It is a type of cancer where the cancer forms in the liver tissues. In hepatoblastoma, the tumour usually forms in the right lobe of the liver. In some instances, hepatoblastoma can spread (advanced or metastasis) and affect other body parts, mainly affecting the lungs. 

Like all other cancers, hepatoblastoma is also staged into four stages. The various stages of hepatoblastoma are:

1. Stage 1 of Hepatoblastoma: Cancer is not spread and can be found outside the liver. Once the tumour is surgically removed, it will be checked under a microscope to see if the margins still have cancer cells.
2. Stage 2 of Hepatoblastoma: In this stage, cancer is not spread outside the liver and is contained within the liver. Once the tumour is removed through a surgical procedure, the cancer cells are found inside the margins upon examination under a microscope.
3. Stage 3 of Hepatoblastoma: Cancer is grown in size and cannot be removed surgically. Cancer may extend into the surrounding liver tissues and the lymph nodes near the liver. The tumours are not found in other body parts.
4. Stage 4 of Hepatoblastoma: This is the advanced stage, where cancer has spread into other organs like the lungs. This stage is also called metastasis. 

The cancer stages help determine the cancer's size and spread. Cancer staging also helps the healthcare provider formulate a proper treatment plan for the patient.

Symptoms of hepatoblastoma become prominent only once the tumour has grown. The hepatoblastoma symptoms differ from one patient to another and may vary depending upon the spread of the tumour and its size. 

It is essential to watch out for the following symptoms of hepatoblastoma:

1. Swelling in the belly (swollen abdomen)
2. A large mass or lump in the stomach (abdomen) may be painful
3. Yellow colour discolouration on the skin and the white part of the eyes is usually due to jaundice
4. A dark-coloured urine
5. Fever
6. Back pain 
7. The appearance of large veins on the skin of the belly
8. Itching on the skin
9. An unusual loss of weight
10. Nausea and vomiting
11. Pain in the abdomen
12. Loss of appetite
13. In many cases, the symptoms of hepatoblastoma may be similar to other health conditions. However, it is essential not to ignore the signs and get a proper diagnosis from the specialist.

The exact causes of hepatoblastoma are not known yet. However, evidence has shown that some of the following conditions can increase the risk of developing hepatoblastoma in children.

1. Premature birth and low birth weight: Babies born prematurely or have a low birth weight are at risk of developing hepatoblastoma.
2. Hepatitis B virus exposure: Children exposed to the hepatitis B virus are also at high risk of developing hepatoblastoma. 
3. Biliary atresia: Biliary atresia is a rare condition seen in newborn babies. In biliary atresia, blockage in the bile ducts prevents bile flow from the liver and into the small intestine. As a result, the bile fluid accumulates in the liver, which damages it. Having biliary atresia increases a child's risk of developing hepatoblastoma.
4. Hemihyperplasia: A rare condition in children that causes one side of the body to grow and become more prominent. This is due to the excessive production of bone tissue.

Certain genetic conditions increase an individual’s risk of developing hepatoblastoma. These genetic conditions that put a child at risk of hepatoblastoma include:

1. Aicardi syndrome is a rare disease in children that affects their brains, eyes, and other body parts, leading to seizures and other disabilities. 
2. Beckwith-Wiedemann syndrome: It is a type of overgrowth disorder.
3. Familial adenomatous polyposis (FAP) is a genetic disorder where precancerous polyps (a small growth) are seen in the colon and rectum.
4. Glycogen storage disease (GSD): It is a rare condition that produces its effect by changing how glycogen is used and stored in the body. 
5. Simpson-Golabi-Behmel syndrome is a condition in which a child grows and gains weight at an unusual pace and looks more prominent than usual. It is usually seen in males and is a type of overgrowth syndrome. 

Generally, one cannot prevent hepatoblastoma. However, specific preventive measures that can help reduce the risk of developing hepatoblastoma include 

1. Getting vaccinated for the hepatitis B virus.
2. Getting timely treatment for chronic hepatitis B infection.
3. Reducing the consumption of food items which are high on aflatoxin B1.

Diagnosis of hepatoblastoma is made based on the following factors:

1. Patient's medical history 
2. The overall health of the patient
3. Age
4. The symptoms they are experiencing

Diagnosing hepatoblastoma also helps determine the cancer stage (the extent of its spread). The various diagnostic tests for diagnosing hepatoblastoma include the following:

1. A complete physical examination and medical history examination.
2. Blood tests: Blood tests like Complete blood count (CBC), serum tumour marker test, liver function tests, and blood chemistry studies will be carried out during the diagnosis of hepatoblastoma. 
3. Alpha-Fetoprotein (AFP) test: This test helps check the levels of Alpha-Fetoprotein (AFP) in the blood for diagnosing hepatoblastoma. This test also helps in preventing the patient's response to the treatment. 
4. Ultrasound: An ultrasound is done to view the patient's abdomen and examine the blood vessels. 
5. A Computerized Tomography (CT or CAT scan): In a CT scan, different angles are used to get several images of the target area within the body. In hepatoblastoma, a CT scan is done for the chest and abdomen. A CT scan may help in viewing the lump formed in the liver and check if cancer has spread to other body parts. 
6. Magnetic Resonance Imaging (MRI) scan: With the help of strong magnets, radio waves, and a computer, a detailed image of the targeted body area can be taken. An MRI scan produces more precise and detailed pictures compared to a CT scan and ultrasound. An MRI scan also helps in viewing the large blood vessels in the liver and checking whether cancer has spread into it.
7. Biopsy: For a biopsy, a sample of the liver tissue is removed by the doctor. This tissue sample is then viewed using a microscope to check for the presence of cancer cells and determine the cancer type. 

How to prepare for the doctor's consultation?

1. Book a prior appointment.
2. Ensure the patient feels comfortable. Wear comfortable clothes.
3. Carry all the past medical records to get a thorough examination done.
4. The doctor will examine the patient physically for the symptoms of hepatoblastoma. 
5. After examining the medical history and completing the physical examination, the doctor will prescribe several diagnostic tests to confirm hepatoblastoma.
6. The patient may ask the following questions to the doctor:
   1. What is hepatoblastoma?
   2. How severe is this cancer?
   3. What are the effects of the disease on the child?
   4. How far is the cancer spread?
   5. Is hepatoblastoma curable?
   6. What are the options available for treating hepatoblastoma?
   7. What surgery will be performed?
   8. What are the side effects to expect from the treatment?
   9. What will the cost of the treatment be?
   10. What does the prognosis say?

Once hepatoblastoma is diagnosed, and the stage of hepatoblastoma is established, the healthcare provider will formulate the treatment plan for the patient. Usually, the treatment for hepatoblastoma depends on the stage of cancer and the symptoms experienced by the patient. If the patient is a child, their age and the number of tumours detected. There are various options available to treat hepatoblastoma. These treatment options to treat hepatoblastoma include the following:

Chemotherapy: Chemotherapy is a treatment method where medicines or drugs destroy cancer cells and stop them from growing. Chemotherapy may be given before or after surgery. 

In chemotherapy, the medication or drug is either given orally or injected into the vein or muscle (systemic chemotherapy). Chemotherapy in hepatoblastoma patients may also be provided by chemoembolisation of the hepatic artery. In chemoembolisation, a thin tube or catheter is used to inject the medicine into the hepatic artery (liver artery). In this procedure, the anticancer therapy is mixed with a substance that cuts off blood flow to the tumour by blocking the artery. This method of regional chemotherapy is also known as transarterial chemoembolisation (TACE). 

Radiation therapy: In radiation therapy or radiotherapy, high-energy X-rays or other types of radiation are focused on the tumour or cancer to destroy them and prevent them from growing. Hepatoblastoma Radiology is also done under this. There are two types of radiation therapy. These include: 

1. External radiation therapy: In this type of radiation therapy, radiation emitted from a machine is used for cancer treatment. A linear accelerator (LINAC) is commonly used to provide external radiation therapy. In this treatment, the high-energy X-rays or electron beams emitted from the machine outside the body are focused on the tumour or cancer cells to destroy them. In patients with hepatoblastoma, external radiation therapy treats tumours that are difficult to remove through surgery or have spread into other body parts.  
2. Internal radiation therapy: In this type of radiation therapy, the radioactive substance is kept in a needle or catheter and placed directly within or close to the cancer. The Radioembolization technique treats hepatoblastoma, where the radioactive substance attached to tiny beads is placed directly into the liver artery through a thin tube or catheter. 

Ablation Therapy: The tumour is destroyed or removed using heat or cool temperature in this treatment method. 

Surgery: Surgery is usually done to remove cancer and may also be done while performing a biopsy. Surgery aims at removing cancer from the liver and providing a cure. The types of surgeries that may be performed depending on the stage of cancer include:

1. Partial Hepatectomy: In this surgical procedure, the portion of the liver where the cancer is found is removed.
2. Total Hepatectomy and a Liver Transplant: This surgical procedure removes the entire liver. After the complete removal of the liver, a healthy liver may be transplanted after a donation from a donor.
3. Metastasis Resection: This surgery is usually performed to remove cancer that has spread to other body parts from the liver, for example, the lungs. 

A child with hepatoblastoma may develop complications as a result of cancer or due to the treatment given. Some of these complications that may arise in a patient having hepatoblastoma include the following:

1. Problems in the heart, kidneys, and lungs
2. Infections 
3. Bleeding and bruising post-surgery 
4. Side effects of chemotherapy like nausea, vomiting, fatigue, weakness, and hair loss
5. Cancer recurrence 
6. Cancer spreading (metastasis)
7. Development of new cancers
8. A new liver transplant if the previous transplanted liver is rejected

When to see a doctor?

A patient must consult a doctor when there is a worsening of the symptoms, fever, or if the side effects of the treatment worsen. If any new symptoms develop, it is essential to see a doctor. 

A doctor prescribes the best diet for patients with hepatoblastoma. Patients with hepatoblastoma are to follow a proper healthy diet, which is essential for speeding up the recovery process. Depending on the patient's overall health, a doctor may prescribe a diet that may include the following: 

1. Eating lots of fruits and vegetables.
2. Consuming whole grains like oatmeal, quinoa, etc.
3. Eating low-fat dairy foods like yoghurt.
4. Eating lean proteins like beans, tofu, eggs, chicken, and fish.
5. Drinking plenty of water and fluids to stay hydrated. 
6. Avoid consuming very spicy, fried, oily, and sweet foods.