Hepatoblastoma

Hepatoblastoma is a kind of liver cancer that usually shows up in babies and little children not older than three years. This is a very rare type of cancer that originates in the liver and doesn’t spread to other body parts. It usually first shows up in the right ear lobe and, if it spreads, you can see it next in the lungs.

Usually, this genetic problem can be first seen in the child’s first 18 months of life. It has been found that white children are affected by this problem more than black children. Also, normally, boys suffer from this more than girls up to the age of five years. Lastly, it is seen in prematurely born babies with low birth weights.

Stages

Hepatoblastoma staging helps determine how far the cancer has spread. The most common system classifies tumours based on their resectability (how much can be removed by surgery), with stages ranging from completely resectable tumours to those with distant metastases. The Children’s Hepatic Tumours International Collaboration (CHIC) system also incorporates factors like AFP levels, patient age, and PRETEXT group to stratify patients into risk groups. 

Here’s a breakdown of the stages:

• Stage I: Tumour is completely resectable by surgery. 
• Stage II: Tumour is mostly resectable, but microscopic amounts of cancer may remain. 
• Stage III: Tumour is not completely resectable at diagnosis, and may involve lymph nodes. 
• Stage IV: Cancer has spread to other parts of the body (metastasis). 

Other staging systems and factors:

• PRETEXT: A system based on pretreatment imaging (CT scans, MRI) to assess tumour size, invasion of vessels, and distant spread. 
• POSTTEXT: A system that classifies tumours postoperatively based on their resectability and residual disease. 
• CHIC: A newer system that incorporates PRETEXT findings, metastases, AFP levels, age, and other factors to stratify patients into risk groups. 
• Recurrent: The disease has returned after treatment. 

Causes

A change in a gene causes this cancer, although not much is known about it, nor is the reason for it understood. Genetic health problems increase a young child’s risk for hepatoblastoma. These are:

• Aicardi Syndrome – Partial or complete absence of the corpus callosum in the brain
• Beckwith-Wiedemann Syndrome – Large birth weight or overgrowth of one side of the body
• Familial Adenomatous Polyposis – These are non-cancerous polyps that families develop in the colon that later become cancerous
• Glycogen Storage Diseases – Rare conditions that alter the way the body uses and stores glycogen
• Babies with low birth weight
• Hemihyperplasia – A condition in which one side of the body or a part of one side is larger than the other. 
• Simpson-Golabi-Behmel Syndrome – A rare genetic disorder that causes overgrowth and various physical abnormalities. 
• Certain genetic changes, such as trisomy 18, also known as Edwards syndrome, are a genetic disorder caused by the presence of a third copy of chromosome 18 in some or all of a person’s cells.

Symptoms

Symptoms include:

• Anemia
• Jaundice
• Distended abdomen
• Stomachache
• Fever
• Loss of appetite
• Back pain
• Itchy skin
• Weight loss
• Vomiting

Diagnosis

When a child complains of a stomachache or a lump in the stomach, the doctor will examine him and treat him after doing the following tests:

• Blood tests
• Ultrasound
• CT scan
• MRI
• Tumor biopsy
• Serum tumour marker test
• SMARCB1 gene testing
• Complete blood count (CBC)
• Liver function tests
• Chest x-ray
• Immunohistochemistry

Treatment

Treating a child for hepatoblastoma is done at various levels. They are:

• Surgery: This helps remove the tumour. If the liver is affected entirely, a liver transplant may be needed.
• Chemotherapy: Here, chemo is injected into the child’s bloodstream, and it travels all over the body. These powerful medicines kill all cancer cells. They can be given either before or after surgery, either intravenously or orally. They can be given straight into the liver too, using a catheter into the organ’s artery.
• Radiation therapy: These are best explained as very powerful X-rays that play a role in killing cancer cells or preventing them from developing further.
• Clinical trials: Your child’s healthcare provider should be able to tell you if there are certain treatments being tested that could suit your little one.
• Supportive care: Whereas treatment can lead to side effects, medicines and other forms of treatment can give pain, infection, fever and nausea.

Who Treats Hepatoblastoma?

Hepatoblastoma, a rare liver cancer primarily affecting children, is treated by a multidisciplinary team of specialists. This team typically includes paediatric oncologists, surgical oncologists, gastroenterologists, radiation oncologists, and supportive care staff, including nurses, nutritionists, and social workers. If a liver transplant is needed, transplant physicians and surgeons are also involved. 

Progressive and Recurrent Hepatoblastoma

Hepatoblastoma may progress or become resistant to treatment. Progressive hepatoblastoma means that the cancer continues to grow, spread, or worsen. Refractory hepatoblastoma is a cancer that no longer responds to treatment.

Recurrent hepatoblastoma refers to cancer that returns after previous treatment. The cancer may come back in the liver or as metastatic tumours in other parts of the body. Tests can help identify the location of the recurring cancer, whether it has spread, and the extent of its progression. The treatment approach for your child’s recurrent hepatoblastoma will depend on the extent of its spread.

Complications

Cancer treatments for hepatoblastoma may cause late effects. These health issues can surface months and years after diagnosis or treatment. Your child may need long-term follow-up and medical care to manage them.

A late effect can result in second cancers, which are new types of cancer. It appears months or years after your child completes hepatoblastoma treatment.

Late effects may make an impact on your child’s:

• Ability to think, learn and remember information
• Growth and development
• Moods, feelings, and mental health
• Organs and tissues

Frequently Asked Questions