What is hepatoblastoma?
Hepatoblastoma is the most mutual liver cancer of childhood, while it affects only 2 to 3 people in a million. It affects children during the first three years of life and usually presents as an abdominal mass that causes pain and discomfort.
They are classified into two types:
“Epithelial type” and “Mixed epithelial/mesenchymal type”. Personalities with familial adenomatous polyposis (FAP), a syndrome of early-onset colonic polyps and adenocarcinoma, frequently develop hepatoblastomas.
Causes of hepatoblastoma
Although the exact cause of liver cancer is unidentified, there are a number of genetic conditions related to an increased risk of developing hepatoblastoma, including the following:
- Beckwith-Wiedemann syndrome
- Familial adenomatous polyposis
- Type I glycogen storage disease
- Alpha-1 antitrypsin deficiency
Children who have a hepatitis B infection at a young age, or those who have biliary atresia (a blockage in the ducts that carry bile), are also at increased risk of developing hepatoblastoma.
What are the signs and symptoms of hepatoblastoma?
A child with hepatoblastoma will usually have a painless mass (a large, protruding belly).
Less common symptoms include:
- Jaundice (yellow skin or eyes)
- Dark urine
- Back pain
- Large veins that can be seen on the skin around the abdomen
- Loss of appetite
- Weight loss
- Nausea and vomiting
Who gets hepatoblastoma
Children are more likely to develop hepatoblastoma if they:
- Are born early with a very low birth heaviness
- Have a growth disorder called Beckwith-Weidman syndrome (which causes the body and internal organs to grow too large or too large)
- Have a genetic disease called familial adenomatous polyposis (which causes growths in the lining of the large intestine)
- Have a problem with a gene (the APC suppressor gene) that normally prevents the tumor’s from growing
Hepatoblastoma is more common in boys than in girls.
Finally, the diagnosis is confirmed by a biopsy of the tumor. Hepatoblastoma can spread to the lungs and rarely to the bones. Therefore, a complete study is necessary at the time of diagnosis to define the extent of the disease. After all the tests are completed, doctors will be able to outline the best treatment options.
In adding to a complete medical history and physical examination, diagnostic actions for hepatoblastoma may include:
- Biopsy: A sample of tissue that is removed from the tumor and examined under a microscope; Your child’s surgeon can also look at the liver using an instrument called a laparoscope, a small tube with a light on the end.
- Complete blood count (CBC): A measure of the size, number, and maturity of different blood cells in a specific volume of your child’s blood.
- Additional blood tests: Blood chemistry, evaluation of liver and kidney functions, and genetic studies.
- Alpha-fetoprotein (AFP) test: Alpha-fetoprotein (AFP) levels in the blood can be used to diagnose and monitor your child’s response to treatment.
Multiple imaging studies, including:
- X-rays: A diagnostic test that uses invisible electromagnetic energy rays to produce images of inside tissues, bones, and organs onto film.
- CT scan (also called a CT scan or CT scan): A CT scan shows detailed images of any part of the body, including bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
- Magnetic Resonance Imaging (MRI): A diagnostic process that uses a combination of large magnets, radiofrequencies, and a computer to produce thorough images of organs and structures within the body.
- Ultrasound (also called ultrasound): Ultrasounds are used to view the function of internal organs and to evaluate blood flow through various vessels.
- Liver scans: Pictures or x-rays taken after injecting a dye that is absorbed by liver tissue. They are used to detect liver tumors and abnormalities.
After completing all the necessary tests, our experts meet to review and discuss what they have learned about your child’s condition. Then we will meet with you and your family to argue the results and outline the best possible treatment options.
How is hepatoblastoma treated?
Specific treatment for hepatoblastoma will be determined by your child’s doctor based on some or all of the following factors:
- Your child’s age, general health, and medical history
- The extent of the disease
- Your child’s tolerance for exact medications, procedures, and therapies
- How your child’s doctor imagines the disease to progress
- Your opinion or preference
Hepatoblastoma is often quite responsive to chemotherapy, so treatment generally, involves both chemotherapy and surgical resection of the tumor.
Most children diagnosed with hepatoblastoma require chemotherapy treatment. Chemotherapy works by interfering with the ability of cancer cells to grow or reproduce.
- Chemotherapy enters the bloodstream and then travels throughout the body to kill cancer cells.
- Different groups of chemotherapy drugs work in different customs to fight cancer cells and shrink tumors.
- Often a grouping of chemotherapy drugs is used.
Although chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. Because of this, there can be many opposing side effects during treatment. Being able to anticipate these side effects can help the care team, the parents, and the child to prepare and, in some cases, prevent these symptoms from occurring. Dana-Farber / Boston Children’s is fully equipped to treat chemotherapy-related symptoms. Each child is assigned a comprehensive team of providers, including a primary care physician, an elementary trainee or trainee, a primary care nurse, and a psychosocial provider.
For patients with a new diagnosis of hepatoblastoma, we offer registration in the most present collaborative Children’s Oncology Group consortium trial. Two of our physicians, Allison O’Neill, MD and Christopher Weldon, MD, Ph.D., were actively involved in the development of the most recent international trial on liver tumors titled PHITT: Pediatric Liver Malignancy International Therapeutic Trial. This trial will study approaches to reduce the intensity of treatment for patients with low-risk disease and increase the intensity of treatment for those with more aggressive disease. The trial will also collect and analyze tumor samples from enrolled patients in hopes of identifying unique therapies.
For patients with recurrent hepatoblastoma who do not respond to chemotherapy, we have experience using:
- Anti-angiogenic drugs that interfere with blood vessel formation
- Targeted agents that disrupt genetic pathways important for tumor growth
- Immunotherapies that take advantage of the patient’s own immune system
Our use of interventional techniques to deliver drugs directly to the tumor and experimental therapies to provide novel therapies for advanced disease sets us apart from other programs.
In addition to medical therapies, surgery is an essential component of therapy for all liver tumors. To remove a tumor, surgery may involve removing part of the liver (partial hepatectomy) or, in rare cases, the entire liver (total hepatectomy), replacing it with a liver from an organ donor (liver transplant). On even rarer occasions, multiple organs may require a transplant (multi-visceral transplant).
Surgery involving the liver is extremely complex and requires a surgeon well versed in this specialized skill. Because liver tumors in children are rare, few surgeons see enough cases to develop extensive experience. At Dana-Farber / Boston Children’s, our experts specialize in pediatric liver surgery and have successfully performed some of the most challenging liver operations in children. In perfect collaboration with Boston Children’s, the nation’s top-ranked pediatric hospital, we have created a multidisciplinary team for each patient that draws from our divisions of oncology, pediatric gastroenterology, interventional radiology, and surgery.
Patients from all over the world choose us for their care. In the last 15 years, our surgeons have performed almost 50 liver tumor surgeries, a large number given the rarity of these diseases. Since 2001, we have performed 25 liver transplants and 3 multi-visceral (multiple organs) transplants for patients with liver tumors. Our advanced capabilities include:
- Autotransplantation: Removal of the liver to allow dissection of an advanced tumor from the organ, while the remaining liver is replaced after the cancer is removed
- Reconstruction of blood vessels or bile ducts
- Heart-lung bypass to eliminate a tumor that has grown into the heart
Very few sites can offer conventional surgery, transplantation, and the support staff needed to care for unique complex cases.
Interventional radiology refers to techniques that allow therapy to be delivered directly to the site of the disease. In the case of liver tumors, interventional procedures may involve:
- Radiofrequency ablation: the destruction of tumors by direct supply of heat
- Transarterial embolization: interruption of blood supply to the tumor
- Transarterial chemoembolization: direct delivery of chemotherapy to the tumor
- Yttrium-90: direct delivery of a radioactive multiple to the tumor
These measures can prevent tumors from growing and/or shrinking the tumors as a bridge to surgery. While these procedures have been available to adult patients for years, they have not yet been used routinely for pediatric patients with liver tumors. We are one of the few pediatric centers in the United States capable of offering these specialized therapeutic approaches to our patients.
- We work in conjunction with a comprehensive radiation oncology program in conjunction with Brigham and Women’s Hospital.
- We offer remote consultations through online second opinion platforms, such as Grand Rounds and MORE Health, reaching patients and families in all corners of the world.