Follicular dendritic cell sarcoma (FDCS) is a rare, slow-growing sarcoma (bone and soft tissue cancerA disease where abnormal cells split without control and spread to other nearby body tissue and/or organs. Cancer cells can also spread to other parts of the body through the bloodstream and lymph systems.) that commonly develops in the lymphA clear fluid that moves through the body through the lymphatic system, carrying cells that fight infection. nodes. More specifically, it develops in dendritic cells
• the smallest, living parts of the body. Cells work together to form or build the body
• a human is made up of millions of Cells
• Cells reproduce themselves to make sure a body stays working
• sometimes Cells can be abnormal or damaged and these can be cancer cells
, which play a vital role in immune function.
There are two types of FDCS, which are categorised by where they originate. The most common type is nodal FDCS, which develops in the dendritic cells in the lymph nodes. This variant is most commonly diagnosed in cervical and axillary (arm pit region) lymph nodes. The less common type is called extranodal FDCS, which develops outside of the lymph nodes. This variant can develop in areas such as the head and neck, digestive systemHow we eat and process foods. Includes organs from: 1. upper gastrointestinal area such as oesophagus, stomach, small intestine, gall bladder, pancreas and liver 2. colorectal area such as colon (big bowel/large intestine), rectum and anus., spleen, liver, lungs, skin, and mediastinum (area between the lungs).
FDCS is most commonly diagnosed in people over 50; however, anyone of any age and gender can develop this disease.
Treatment
If FDCS is detected, it will be staged and graded based on size, metastasisKnown as secondary cancer, it grows/spreads from the original/ primary cancer. (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 localisedOnly to one area/place 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:
- Grade
• A score that tells how quickly a tumour might spread and grow by looking at how the abnormal cells and tissue look under a microscope.
• Grade is not the same as stage.
• Grading is different depending on the type of cancer.
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 FDCS may include:
- Surgery to remove as much of the tumour as possible.
- Radiation therapy.
- Chemotherapy
• a chemical drug treatment to kill or slow-growing cancer Cells
• these drugs are called cytotoxic drugs
. - Targeted therapies.
- Clinical trials.
- Palliative careLessening pain without curing the disease..
For more information on treatment options, please refer to the Rare Cancers Australia treatment options page.
Risk factors
Because of how rare FDCS is, there has been minimal research into the risk factors of this disease. However, some conditions have demonstrated a potential link to the development to FDCS, including:
- Castleman’s disease.
- Epstein Barr virus.
- Paraneoplastic pemphigus.
- Myasthenia gravis.
Not everyone with these conditions will develop the FDCS and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.
Symptoms
In the early stages of FDCS, symptoms are often absent or confused for other conditions. As the cancer progresses, symptoms may appear but will vary depending on location. Some of these symptoms may include:
- Painless swelling, inflammation and/or lump in lymph nodeA small lump or mass of tissue in your body.A small lump or mass of tissue in your body.A small lump or mass of tissue in your body. (nodal FDCS) or elsewhere (extranodal FDCS).
- Fever.
- Unexplained weight loss.
- Abdominal pain that worsens as abdominal massA growth of cells that come together to make a lump, either big or small, and may or may not be cancer. grows.
- Cough and/or sore throat.
- Fatigue.
- Night sweats.
Not everyone with the symptoms above will have cancer but see your GP if you are concerned.
Diagnosis/diagnosing
FDCS is often misdiagnosed as other conditions. If your doctor suspects you have FDCS, they will order a range of diagnostic tests to confirm the diagnosisUsing medical test results, identify and name a disease and/or condition. and refer you to a specialist for treatment.
Imaging & blood tests
The doctor will take images of your body using magnetic resonance imagingTaking images/photos of inside body parts using magnet rather than x-ray. (MRITaking images/photos of inside body parts using magnet rather than x-ray.), a computed tomography scan (CT scanA 3-D (three dimensional) x-ray pictures that gives more information than a normal x-ray.), x-ray, and/or positron emission tomography (PET scanA test that uses a radioactive drug to show a picture of how your tissues and organs are working. Also known as a positron emission tomography 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 bloodhello test may be taken to assessTo measure, look at and learn from. your overall health and help guide treatment decisions.
Biopsy
Once the location(s) of the cancer has been identified, the doctor will perform a biopsyTo take a small piece of body tissue and test it in a laboratory. to remove a section of tissue using a needle. The tissue sample will then be analysed for cancer cells.
