Hepatoblastoma - Diagnosis and Treatment

Introduction

Hepatoblastoma is an aggressive form of liver cancer that mainly affects small children, usually under the age of three. Though it is a rare type of childhood cancer, it is very challenging to manage when it occurs. Advances in diagnosing and treating this have opened a wide ray of hope for affected children and families. This article explores hepatoblastoma, its diagnosis, and treatment in detail.

What Is Hepatoblastoma?

It is a rare form of tumor originating in the liver cells and has the potential for rapid growth. It is primarily diagnosed in infants and young children, usually under three. It is not common in adults. The cause of this is generally unknown. Certain genetic risk factors have been involved. Premature babies with low birth weight are at higher risk of developing this tumor. Children who are suffering from hepatoblastoma present with swelling and pain in the abdomen, loss of hunger with significant weight loss, and jaundice. The exact cause of hepatoblastoma is primarily genetic predisposition and syndromes like Beckwith-Wideman syndrome. They are also associated with exposure to certain chemicals such as arsenic, cadmium, and nicotine that may contribute to development.

How to Diagnose Hepatoblastoma?

Imaging Techniques:

• Ultrasonography: This is the most commonly employed imaging technique for initial diagnosis. It is less expensive and noninvasive, helping detect any liver masses. It reveals a large liver mass with areas of bleeding and satellite lesions within it.

• Computer Tomography (CT) and Magnetic Resonance Imaging (MRI): These techniques provide advanced images with detailed information if any mass is present in relationship with adjacent structures that can be visualized. Hypervascular lesions were seen in the liver with a delay in the extraction of contrast agents. Chest and brain CT were needed for formal staging.

Histological Examination:

A biopsy is the diagnostic tool of choice to confirm most tumors. In hepatoblastoma, a needle biopsy guided with the help of imaging is utilized to collect the samples. On histological examination, the tumor can be divided into stages, and the tumor can be further studied.

• Blood Tests: This is done using tumor markers like Alpha-fetoprotein. This marker has been associated with hepatoblastoma; this increased serum level is a confirmatory diagnosis. This helped in diagnosis and monitoring the progress of treatment and reference.

• Risk Stratification: This is done as a pretext and posttext group. Pretest is assigned at the time of diagnosis and posttext after treatment. Based on the advancement and number of lobes involved, they are numbered using Roman numerals.

What Are the Multimodal Treatments Available?

Multimodal therapy includes complete resection of the liver along with transplantation and chemotherapy, based on the extent of the tumor involved.

• Surgery: Surgical resection is usually the primary treatment indicated where a partial or complete hepatectomy (liver removal) is carried out. These can also be used to take a sample for diagnosis. This mode of treatment aims to remove the tumor by preserving healthy liver tissue as much as possible.

• Chemotherapy: Cancer-killing medicines are given to the child before or after surgery. They are either given intravenously or orally. They can also be injected directly into the liver, this method is known as chemoembolization. This medicine blocks the blood supply to the tumor and stops its growth. Neoadjuvant chemotherapy includes platinum-based regimens that utilize medicines like Cisplatin and Doxorubicin. They shrink and reduce the size of the tumor, favoring its resection.

• Transplantation: This is the last reason when all other methods fail; when unable to resect, the tumor total liver has to be removed, and a transplant taken from the donor is used as a replacement. Advances in transplantation techniques have significantly increased the survival rates and improved patient outcomes.

• Targeted Therapy: This focuses on molecules to enhance the treatment outcomes. Though it seems very promising, its effect varies from patient to patient. New treatment modalities and combinations are emerging. They include kinase inhibitors targeting receptors for tumor growth and behave as immune checkpoints. Examples are Cabozantinib, Nivolumab, and Sorafenib.

• Radiotherapy: It is not considered a standard treatment mode in hepatoblastoma, as there might be long-term side effects on the growing liver. It is employed in cases where it is difficult to resect or, there is a high chance of recurrence. When used, advanced techniques are advocated; they are

  • Image-Guided Radiation Therapy: Radiation delivery is very precise with the help of imaging using CT or MRI.

  • Intensity-Modulated Radiation Therapy: This technique adjusts the radiation beam's intensity, which makes the radiation more targeted and spares the risk of affecting healthy tissues.

• Supportive Care: The children undergoing treatment have a lot of support. They may have trouble eating and be tired. So, the caretaker has to ensure proper nutrition and adequate rest. The point is to manage symptoms and make life easier. Also, there is a need to reduce the side effects of treatment as much as possible. Apart from the caretaker, a multidisciplinary team including a pediatrician, oncologist, nurses, nutritionist, and social worker should all collaborate and provide the child with emotional and psychosocial support.

What Are the Challenges Faced?

Since it is mostly in young children, it is very challenging to make them undergo treatment. There is also a need to manage the treatment-related side effects, optimize the treatment outcomes, and address the parents' concerns about recurrence. To have a better outcome, detecting the disease early on is necessary. Increasing awareness and implementing screening in high-risk populations helps in this regard.

Conclusion

Advances in the medical world have significantly improved treatment outcomes for children affected with hepatoblastoma. A multimodal treatment modality, comprising surgery, liver transplantation when needed, and chemotherapy, has become the standard of care. Further research is ongoing exploring targeted therapies and immune therapies. Early diagnosis using tumor markers, imaging, and blood work considerably helps improve survival chances. Apart from early and accurate diagnosis, a personalized treatment plan is important for better outcomes. With the current advancement in diagnostics and treatment aspects, the medical team is ready to enhance further the quality of life and long-term survival of children suffering from hepatoblastoma and instill hope in their families.