What is hepatocellular carcinoma?
Hepatocellular carcinoma, also called liver carcinoma, is a rare, aggressive type of liver cancer. There are two predominant subtypes of hepatocellular carcinoma: conventional and fibrolamellar.
- Conventional hepatocellular carcinoma is more frequently diagnosed in adults and is often seen in conjunction with underlying liver disease (for example, cirrhosis). When diagnosed in children, hepatocellular carcinoma may be linked to hepatitis infection or genetic conditions. However, it often occurs in the context of a normal, healthy liver and is fundamentally felt to be a disease unique to that seen in adults.
- Fibrolamellar hepatocellular carcinoma is a unique, rare entity apart from conventional hepatocellular carcinoma and requires novel and experienced approaches to care.
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.
Hepatocellular carcinoma traditionally affects children in adolescence and young adulthood. Rarely, it can occur in very young children.
What causes hepatocellular carcinoma in children?
Although the exact cause of hepatocellular carcinoma is unknown, it can occasionally occur in children with an underlying cause of liver dysfunction such as:
- chronic infection
- autoimmune hepatitis
- biliary atresia
- glycogen storage diseases
- alpha-1 antitrypsin
What are the symptoms of hepatocellular carcinoma 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 a swollen abdomen
- pain on the right side that may extend to the back and shoulder
- back pain from compression of the tumor
- decreased appetite and weight loss
- itching skin
How we care for hepatocellular carcinoma at Dana-Farber/Boston Children's
Children with hepatocellular carcinoma 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.
Hepatocellular Carcinoma | Diagnosis & Treatments
How is hepatocellular carcinoma diagnosed?
Hepatocellular carcinoma can spread to the lungs, lymph nodes within the abdomen, the lining of the abdominal cavity, 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 hepatocellular carcinoma may include:
- complete blood count (CBC)
- additional blood tests
- alpha-fetoprotein (AFP) test
- computerized tomography scan
- magnetic resonance imaging (MRI)
After we complete all necessary tests, our team of 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 discuss the results and outline the best possible treatment options.
How is hepatocellular carcinoma treated?
Your child’s physician will determine the specific treatment for hepatocellular carcinoma, 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 physician expects the disease to progress
- your opinion or preference
Patients require both systemic therapy (such as chemotherapy) and local control, or surgery, to achieve cure. Hepatocellcular carcinoma is only variably responsive to chemotherapy. Given the rarity of this disease, both conventional and fibrolamellar, your child should be treated at a large referral center that sees a number of these cases per year.
In some instances, children diagnosed with hepatocellular carcinoma 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.
Hepatocellular carcinoma is often less responsive to chemotherapy than hepatoblastoma, the most common liver tumor in children. Because of this, surgical resection of disease is crucial. For patients with recurrent hepatocellular carcinoma unresponsive to chemotherapy, we offer a wide range of alternative therapies:
- antiangiogenic drugs that interfere with the formation of blood vessels
- targeted agents that interrupt genetic pathways important for tumor growth
- immunotherapies that capitalize on the patient’s own immune system
In addition to medical therapies, surgery is a necessary component of therapy for all liver tumors, particularly hepatocellular carcinoma. 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. 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 more than 120 liver resections and more than 35 liver transplants, as well as three 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
- advanced imaging techniques (MRI with eovist, MRCP, PET/CT)
- interventional radiology procedures
Surgery may be more difficult for patients with hepatocellular carcinoma, as this type of cancer has often spread by the time it is diagnosed, can involve multiple parts of the liver, and can occur in patients with underlying liver disease. For this reason, a highly skilled, multidisciplinary care team familiar with the care of these challenging scenarios is crucial.
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 U.S. able to offer these specialized therapeutic approaches to our patients.
We collaborate with Brigham and Women’s Hospital to provide our patients with comprehensive radiation therapy oncology when necessary.
Our Institutional Experimental Therapeutics group offers novel therapies for patients with relapsed or refractory disease.