Fibrolamellar Carcinoma (FLC/FLHCC)
What is fibrolamellar carcinoma?
Fibrolamellar carcinoma, or FLC, is a rare cancer of the liver that usually grows in teens and adults under 40 years old. This type of cancer is different than other types of liver cancer because it happens in people who have healthy livers. Other liver cancers tend to grow in people with livers that have been damaged by alcohol abuse or infection with certain viruses. Fibrolamellar carcinoma may be called by many different names, including eosinophilic glassy cell hepatoma, fibrolamellar oncocytic hepatoma, fibrolamellar hepatocellular carcinoma (FLHCC) or FHCC.
How common is fibrolamellar carcinoma?
FLC is so rare that there is little data on how many people have it. It is thought to make up 1% to 5% of all liver cancers. FLC affects both men and women and is thought to occur in about one in five million people in the US.
How is fibrolamellar carcinoma diagnosed?
Many people with FLC do not have symptoms when the cancer first starts. Later, when the tumor gets larger, symptoms can include:
- Pain in the abdomen, shoulder, or back
- Nausea and vomiting
- Appetite and weight loss
- Malaise (having less energy or just feeling unwell)
- Jaundice (yellowing of the skin)
Imaging: If you have symptoms of FLC, your doctor will use imaging scans such as CT, MRI, and ultrasound to look at the size of the tumor and where it is. They will also check for signs that the tumor has spread to other parts of the body.
Biopsy: To check if the tumor is FLC, your doctor will perform a biopsy, taking a small sample from the tumor with a needle. An expert, called a pathologist, will study cells from the sample under the microscope to see what kind of tumor it is.
How is fibrolamellar carcinoma treated?
Surgery: Surgery is used to remove as much of the FLC as possible. This surgery can involve either a resection, where part of the liver is taken out, or a transplant, where the whole liver is taken out and replaced with a donor liver. If the surgeon does a resection, you will be able to live and function well even with part of your liver missing.
Chemotherapy: When surgery is not possible or when the cancer has spread, chemotherapy will be used to treat the FLC.
Embolization Therapy: Depending on where the FLC is, embolization therapy can be used. This type of therapy cuts off the blood supply to the part of the liver where the FLC is located. This causes the FLC to die because it can’t get oxygen and nutrients.
Does fibrolamellar carcinoma run in families?
Because there are so few people with FLC, we do not know if it runs in families.
How does fibrolamellar carcinoma form?
Scientists are always working to understand how cancer forms, but it can be hard to prove. We know that in FLC, a chromosome (the structures in your cells that contain all of your genes) breaks apart and gets put back together in the wrong way. This can cause cells to not function like they should. In FLC, a gene called DNAJB1 joins with a gene called PRKACA. This happens in almost all cases, so it may be very important for how FLC forms. Scientists are trying to figure out how this works, so they can invent new therapies. Your doctor may test your tumor for DNAJB1 and PRKACA to confirm it is FLC.
What is the prognosis for someone with fibrolamellar carcinoma?
The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as:
- Where the tumor is in your body
- If the cancer has spread to other parts of your body
- How much of the tumor was taken out during surgery
If you want information on your prognosis, it is important to talk to your doctor. NCI also has resources to help you understand cancer prognosis.
Doctors estimate survival rates by how people with FLC have done in the past. Because there are so few cases, these rates may not be very accurate. They also don’t consider that newer treatments are being developed.
We do know that surgery is very important for surviving longer. For people with FLC who are treated with surgery, 44% to 68% survive for five years. When people with FLC get other treatment without surgery, 2% to 17% survive for five years.
New understanding of chromosome changes in FLC may help find better treatments for this tumor.