What is hepatoblastoma?
- Hepatoblastoma is a rare type of childhood cancer that occurs in the liver.
- The primary functions of the liver include filtering and storing blood, processing the food we eat, and making vital proteins for the body to use.
- Hepatoblastoma traditionally affects children from infancy to about age 5; most cases appear during the first 18 months of life.
What causes hepatoblastoma in children?
Although the exact cause of hepatoblastoma is unknown, children who have been born prematurely, are of low birth weight, or who have genetic condition (including but not limited to hemihypertrophy syndromes, Beckwith-Wiedemann, trisomy 18, and germline APC gene mutations) are at increased risk of developing hepatoblastoma.
What are the symptoms of hepatoblastoma in children?
Different children may experience symptoms differently, and symptoms may vary depending on the size of the tumor and whether the tumor has spread.
Your child's symptoms may include:
- a large abdominal mass, or swollen abdomen
- weight loss, decreased appetite
- abdominal pain
- vomiting
- jaundice (yellowing of the eyes and skin)
- fever
- itching skin
- anemia (pale skin and lips from a decreased number of red blood cells)
- back pain from compression of the tumor
How we care for hepatoblastoma
Children with hepatoblastoma are treated in the Liver Tumor Center at the Dana-Farber/Boston Children’s Cancer and Blood Disorders Center. We have some of the most experienced pediatric liver cancer oncologists and surgeons in the world, as well as internationally recognized pediatric subspecialists who use sophisticated technology and therapies to maximize outcomes for our patients.
Hepatoblastoma | Diagnosis & Treatments
How is hepatoblastoma diagnosed?
Hepatoblastoma can spread to the lungs, lymph nodes within the abdomen, and rarely to the bone or brain. For this reason, a full work-up is necessary to define disease extent.
In addition to a complete medical history and physical examination, diagnostic procedures for hepatoblastoma may include:
- biopsy
- complete blood count (CBC)
- additional blood tests
- alpha-fetoprotein (AFP) test
- x-ray
- computerized tomography scan (also called a CT scan)
- magnetic resonance imaging (MRI)
- ultrasound
After we complete all necessary tests, our team will meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best possible treatment options.
How is hepatoblastoma treated?
Your child’s physician will determine the specific treatment for hepatoblastoma, based on some or all of the following factors:
- your child's age, overall health, and medical history
- the appearance of your child’s tumor under the microscope
- extent of the disease
- your child's tolerance for specific medications, procedures, and therapies
- how your child's physicians expect the disease to progress
- your opinion or preference
Patients require both systemic therapy (e.g. chemotherapy) and local control, or surgery, to achieve cure. Hepatoblastoma is often quite responsive to chemotherapy.
Chemotherapy treatment for hepatoblastoma
Most children diagnosed with hepatoblastoma require treatment with chemotherapy. Chemotherapy works by interfering with the cancer cells’ ability to grow or reproduce. Chemotherapy is introduced to the bloodstream and then travels throughout the body to kill cancer cells, different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors. Often, a combination of chemotherapy drugs is used.
While 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 adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent, these symptoms from occurring. Each child is assigned a comprehensive team of providers, which includes a primary attending physician, a primary fellow or trainee, a nurse practitioner, a primary nurse, and a psychosocial provider.
Surgery
In addition to medical therapies, surgery is a necessary component of therapy for all liver tumors, including hepatoblastoma. Surgery may involve removing a portion 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 transplantation (multivisceral transplant).
Surgery involving the liver is extraordinarily complex and requires a surgeon well-versed in this specialized skill. Surgery involving the liver is extraordinarily complex and requires a surgeon well-versed in this specialized skill. Because liver tumors in children are rare, it is crucial that liver surgeries be performed by surgeons with unique and deep expertise in liver tumor resections. Our experts specialize in pediatric liver surgery and have successfully performed some of the most complicated liver operations in children.
Within the past 20 years, our surgeons have performed over 120 liver resections and over 35 liver transplants as well as 3 multivisceral transplants for patients with liver tumors.
We offer the most advanced and aggressive surgical techniques available including:
- transjugular and conventional interventionally guided liver biopsies
- ex vivo resection with autotransplantation
- blood vessel or bile-duct reconstruction
- heart-lung bypass
- use of indocyanine green (ICG)
- Advanced imaging techniques (MRI with eovist, MRCP, PET/CT)
- Interventional radiology procedures (TACE, Y-90)
There are very few sites in the United States that can offer these procedures.
Interventional Radiology
Interventional radiology refers to techniques that allow delivery of therapy directly to the site of disease. In the case of liver tumors, interventional procedures can involve:
- destruction of tumors through direct delivery of heat
- disruption of the blood supply to the tumor
- direct delivery of chemotherapy to the tumor
- direct delivery of a radioactive compound to the tumor
These measures can prevent tumors from growing and shrink tumors as a bridge to surgery. While these procedures have been available to adult patients for years, they have yet to be routinely used in children with liver tumors. We are one of the few pediatric sites in the United States able to offer these specialized therapeutic approaches to our patients.
Radiation
We collaborate with Brigham and Women’s Hospital to provide our patients with comprehensive radiation therapy when necessary.
Relapsed disease
Our Institutional Experimental Therapeutics group offers novel therapies for patients with relapsed or refractory disease.
