Fibrolamellar carcinomacancer arising from tissues that line organs (FLC), or fibrolamellar hepatocellular carcinoma (FL-HCC or FHCC), is a rare form of cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs 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 chronica long-lasting disease that changes slowly over time 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 prognosisto predict how a disease/condition may progress and what the outcome might be than HCC.
Treatment
If FLC is detected, it will be staged and graded based on size, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancer cellsthe basic structural and functional unit of all living things look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumoura tissue mass that forms from groups of unhealthy cells) indicates the size and depth of the tumour.
- N (nodea small lump or mass of tissue in your body) indicates whether the cancer has spread to nearby lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid.
- 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 tissuea group of cells that work together to perform a function.
- 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 localisedaffecting only one area of body 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 advancedat a late stage, far along or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells 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 testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, 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:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence, potentially including:
- Tumour resectionsurgical removal of tissue or part/all of an organ.
- Hepatectomycomplete or partial removal of the liver.
- Organ transplanta surgical procedure that involves removing a failing or damaged organ and replacing it with a healthy one from a donor (liver).
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Therapeutic embolisationa treatment that aims to reduce blood flow to the cancer and ultimately reduce cancer growth.
- Immunotherapya treatment that uses a person's immune system to fight cancer.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Risk factors
Because of how rare FLC is, there has been limited research into the riskthe possibility that something bad will happen 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.
- Nauseato feel sick or likely to vomit and vomiting.
- Unexplained weight loss/loss of appetite.
- Fatiguea state of extreme tiredness or exhaustion, can be physical or mental.
- Jaundiceyellowing of the skin and the whites of the eyes.
Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.
Some of the information regarding symptoms was obtained from the Fibrolamellar Carcinoma (FCL/FLHCC) page published by the National Cancer Institute.
Diagnosis
If your doctor suspects you have FLC, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Imagingtests that create detailed images of areas inside the body tests, potentially including:
- MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology to create detailed images of the body.
- Ultrasounda type of medical imaging that uses soundwaves to create detailed images of the body .
- Blood teststesting done to measure the levels of certain substances in the blood.
- Biopsyremoval of a section of tissue to analyse for cancer cells.
