Childhood Hepatoblastoma is a rare form of liver cancer that primarily affects children, typically under the age of 5. It accounts for about 1% of all childhood cancers, making it a relatively rare pediatric condition. Despite its rarity, hepatoblastoma is the most common liver cancer found in children. Early detection and treatment can significantly improve the prognosis, and survival rates are high when the disease is detected early and confined to the liver. (Source)
What is Childhood Hepatoblastoma?
Hepatoblastoma is a type of malignant tumor that originates in the hepatocytes, the primary cells of the liver. These cancerous cells grow rapidly, forming tumors in the liver. Hepatoblastoma typically affects only one lobe of the liver and can sometimes spread to other parts of the body if not treated promptly.
There are two main types of hepatoblastoma:
- Epithelial Type: Composed mainly of liver cells and is the more common type.
- Mixed Epithelial and Mesenchymal Type: Contains both liver cells and mesenchymal (connective tissue) components, which can include bone or cartilage cells. (Source)
How is Childhood Hepatoblastoma Caused?
The exact cause of hepatoblastoma is unknown, but several risk factors are associated with an increased likelihood of developing the disease. These include:
- Premature Birth and Low Birth Weight: Children born prematurely or with a low birth weight are at a higher risk of developing hepatoblastoma.
- Genetic Conditions: Certain genetic syndromes increase the risk of hepatoblastoma, including:
- Beckwith-Wiedemann Syndrome: A growth disorder that predisposes children to various cancers.
- Familial Adenomatous Polyposis (FAP): A genetic condition that leads to the development of numerous polyps in the colon and an increased risk of liver cancer.
- Trisomy 18 (Edward Syndrome): A chromosomal abnormality that is linked to higher cancer risks, including hepatoblastoma.
Other factors such as exposure to environmental toxins, though suspected, have not been definitively linked to the development of this cancer. (Source)
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How Does Childhood Hepatoblastoma Spread?
Hepatoblastoma typically starts in one lobe of the liver. If not treated, the cancer can spread to other parts of the body. The most common sites of metastasis include the lungs, abdominal cavity, and bones. The tumor can also invade blood vessels, spreading cancer cells through the bloodstream to other organs. Early detection and treatment are critical to prevent metastasis.
Symptoms of Childhood Hepatoblastoma
In its early stages, hepatoblastoma may not cause noticeable symptoms, but as the tumor grows, several signs may appear. Common symptoms include:
- Abdominal Swelling or Mass: A swollen abdomen or the presence of a palpable mass is one of the most common signs of hepatoblastoma.
- Pain in the Abdomen: The child may complain of pain or discomfort in the stomach area as the tumor enlarges.
- Weight Loss and Poor Appetite: Unexplained weight loss and a lack of interest in eating may occur.
- Nausea and Vomiting: As the tumor grows and affects liver function, nausea and vomiting may develop.
- Jaundice: Yellowing of the skin and eyes may occur if the tumor disrupts the liver’s ability to process bile.
- Fever: Unexplained fevers can be a sign of liver inflammation due to cancer.
- Fatigue: The child may exhibit unusual tiredness or lethargy. (Source)
Diagnosis of Childhood Hepatoblastoma
Several diagnostic tools and procedures are used to confirm the presence of hepatoblastoma:
- Physical Examination: The doctor may check for signs of abdominal swelling or a lump in the liver area.
- Blood Tests:
- Alpha-Fetoprotein (AFP): This protein is typically elevated in children with hepatoblastoma. A blood test to measure AFP levels can help in diagnosing and monitoring treatment response.
- Imaging Tests:
- Ultrasound: Often the first imaging test used to detect liver abnormalities.
- CT Scan or MRI: These imaging tests provide detailed pictures of the liver and help determine the size, location, and spread of the tumor.
- X-rays: Used to check if the cancer has spread to the lungs.
- Biopsy: A small sample of the tumor is taken and examined under a microscope to confirm the diagnosis.
- Liver Function Tests: These tests help evaluate how well the liver is working and if the tumor is affecting its function.
Treatment of Childhood Hepatoblastoma
Treatment for hepatoblastoma depends on several factors, including the size of the tumor, its spread, and the overall health of the child. The primary treatment options include:
- Surgery:
- Tumor Resection: If the tumor is localized, surgery is often the first treatment option. The goal is to remove the entire tumor along with a margin of healthy tissue.
- Liver Transplant: In cases where the tumor is too large to be removed, a liver transplant may be necessary.
- Chemotherapy:
- Chemotherapy is often used before surgery to shrink the tumor and after surgery to eliminate any remaining cancer cells. Cisplatin and doxorubicin are commonly used chemotherapy drugs in hepatoblastoma treatment.
- Radiation Therapy:
- Radiation is not commonly used in hepatoblastoma treatment, but it may be considered if surgery is not possible or the cancer has spread.
- Targeted Therapy:
- Targeted drugs are being investigated to treat advanced hepatoblastoma, specifically drugs that inhibit the pathways promoting tumor growth.
- Clinical Trials:
- Clinical trials offer access to newer treatments that may not be widely available and are an option for children with advanced hepatoblastoma. (Source)
Can Childhood Hepatoblastoma Be Prevented?
There is no known way to prevent hepatoblastoma, as the exact causes are not fully understood. However, in cases linked to genetic conditions like Beckwith-Wiedemann Syndrome or Familial Adenomatous Polyposis, regular screenings and early detection can improve outcomes. For children with known risk factors, particularly those born with genetic syndromes, close monitoring and routine imaging may help detect hepatoblastoma early when treatment is more effective. (Source)
Is Childhood Hepatoblastoma Hereditary?
In most cases, hepatoblastoma is not hereditary. However, a small percentage of cases are associated with genetic conditions that can be passed down from parents to their children, such as Beckwith-Wiedemann Syndrome and Familial Adenomatous Polyposis. These conditions increase the risk of developing hepatoblastoma and other cancers. Genetic counseling and testing are recommended for families with a history of these syndromes. (Source)
Can Childhood Hepatoblastoma Be Cured?
Yes, childhood hepatoblastoma can be cured, especially when it is diagnosed and treated in the early stages. The 5-year survival rate for children with localized hepatoblastoma who undergo surgery and chemotherapy is 70% to 80%. If the cancer has not spread beyond the liver, the chances of a complete cure are significantly higher. However, if the cancer has metastasized or if the tumor cannot be completely removed, the prognosis may be less favorable.
For children with advanced hepatoblastoma, liver transplants and emerging therapies, including clinical trials, offer potential paths to cure. Early diagnosis and aggressive treatment are critical to improving survival outcomes. (Source)
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Conclusion
Childhood hepatoblastoma is a rare but treatable form of liver cancer. While the exact cause is unknown, certain genetic conditions increase the risk of developing the disease. Early diagnosis, often through physical examination and imaging tests, is key to successful treatment. Surgery, chemotherapy, and, in some cases, liver transplants are the main treatment options. While there is no way to prevent hepatoblastoma, genetic counseling and early screening are important for high-risk children. With early intervention, most children with hepatoblastoma can be cured, and survival rates are high.
