Fibrolamellar carcinoma (FLC), or fibrolamellar hepatocellular carcinoma (FL-HCC or FHCC), is a rare form of cancer that develops from the tissues lining the liver. FLC presents in patients without any prior liver damage, which makes it stand out from most other types of liver cancers.
FLC is considered to be a variant of hepatocellular carcinoma (HCC), which is the most commonly diagnosed primary liver cancer. HCC is generally diagnosed in people over 40 with chronic liver conditions and has male prevalence. Contrastingly, FLC generally affects people under 40 years old who have not had any prior liver damage, and is diagnosed equally in males and females. Generally, FLC also has a better prognosis than HCC.
Treatment
If FLC is detected, it will be staged and graded based on size, metastasis (whether the cancer has spread to other parts of the body) and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumour) indicates the size and depth of the tumour.
- N (nodes) indicates whether the cancer has spread to nearby lymph nodes.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0-IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment is dependent on several factors, including age, stage of disease and overall health. The types of treatments generally don’t vary between adults and children.
Treatment options for FLC may include:
- Surgery, potentially including:
- Tumour resection.
- Hepatectomy (removal of part or all of the liver).
- Liver transplant (in extreme cases).
- Radiation therapy.
- Chemotherapy.
- Therapeutic embolisation.
- Immunotherapy.
- Clinical trials.
- Palliative care.
For more information on treatment options, please refer to the Rare Cancers Australia treatment options page.
Risk factors
Because of how rare FLC is, there has been limited research into the risk factors of this disease.
Early symptoms
Early-stage FLC is difficult to diagnose as there usually aren’t any symptoms. As the tumour progresses, symptoms may begin to appear, including:
- Abdominal, back and/or shoulder pain.
- Nausea and vomiting.
- Unexplained weight loss/loss of appetite.
- Fatigue.
- Jaundice (yellowing of eyes and/or skin).
Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.
Diagnosis/diagnosing
If your doctor suspects you have FLC, they will order a range of diagnostic tests to confirm the diagnosis, and refer you to a specialist for treatment.
Imaging & blood tests
The doctor will take images of your body using magnetic resonance imaging (MRI), a computed tomography scan (CT scan), x-ray, and/or positron emission tomography (PET scan), depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and looks for signs of metastasis. Additionally, a blood test may be taken to assess your overall health and help guide treatment decisions.
Biopsy
Once the location(s) of the cancer has been identified, the doctor will perform a biopsy to remove a section of tissue using a needle. The tissue sample will then be analysed for cancer cells.
Prognosis (Certain factors affect the prognosis and treatment options)
While it is not possible to predict the exact course of the disease, your doctor will be able to give you a general idea based on rate and depth of tumour growth, susceptibility to treatment, age, overall fitness and medical history. Generally, early-stage FLC have good prognosis and survival rates. However, if the cancer is advanced and has spread, the prognosis is not as good and there is a higher risk of cancer recurrence.
References
Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.