Follicular dendritic cell sarcoma
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Follicular dendritic cell sarcoma (FDC sarcoma) is a very rare type of blood cancer. Fewer than 100 cases have been reported in medical literature world wide.
Follicular dendritic cell sarcoma develops from specialised cells in the lymph nodes. Most develop in the lymph nodes and are called nodal cancers. But about 3 out of 10 (30%) develop elsewhere in the body in the
- Head and neck
- Digestive system (bowel, stomach)
These FDC sarcomas are known as extranodal tumours, because they grow outside the lymph nodes and bone marrow.
There are a number of different types of cell in the lymph nodes, including follicular dendritic cells (FDC). FDCs are found in the part of the node called the lymphoid follicle where a type of white blood cell (lymphocyte) is made. Lymphocytes help to fight infection.
Follicular dendritic cells support lymphocytes by recognising cells that are foreign or unwanted in the body. They pick them out by looking at the proteins (antigens) on the cell surface, which are different from proteins on normal cells. The FD cells then capture the foreign cells and take them to lymphocytes. The lymph nodes can then make lymphocytes that recognise the particular cells that the FDC cells have found. The foreign cells can then be picked up quickly and destroyed by the immune system.
We don’t know what causes follicular dendritic cell sarcoma. It is particularly difficult to find out the cause of a rare cancer because there are so few people with the disease. This makes it hard to find a common link.
Doctors have linked follicular dendritic cell sarcoma to Castleman’s disease ̶̶ a condition where benign tumours develop in the lymph nodes. But we need more research to confirm this. We also know from research that the Epstein Barr virus may play a part in the development of FDC sarcomas of the liver and spleen.
The symptoms of follicular dendritic cell sarcoma vary, depending on where in the body it develops. The most common symptom is a painless swelling of a lymph node usually in the neck. But a swelling can develop elsewhere in the lymphatic system. Follicular dendritic cell sarcomas are described as indolent. An indolent cancer is one that grows slowly. Other symptoms may include a
- Sore throat
- Difficulty in swallowing
- Weight loss
The only way to make a definite diagnosis is to take some cells from the lump (a biopsy) to look at under a microscope. Follicular dendritic cell sarcomas are difficult to diagnose because they can look very like other types of cancer, including lymphomas and other types of sarcoma.
Follicular dendritic cell sarcomas have particular proteins on the cells that doctors can test for. The proteins include CD21, CD23 and CD35. This helps to confirm the diagnosis. It is called immunohistochemical staining. Doctors call these proteins markers.
Follicular dendritic cell sarcomas are sometimes a bit similar to lymphomas, but doctors usually treat them as a soft tissue sarcoma. Your doctors will plan your treatment taking into account
- How big your cancer is and if it has spread elsewhere in your body (the stage)
- How different the sarcoma cells are from normal cells and how fast they grow (the grade)
- Your general health and fitness
Doctors most commonly treat sarcomas with the chemotherapy drugs ifosfamide and doxorubicin. You can find out more about other drugs used to treat sarcoma in the section about treating soft tissue sarcoma.
Once you have finished your treatment you will have regular check ups. At first the appointments will be every few months. If all is well, they will become less frequent.
If your cancer comes back or the first treatment does not work your doctor may suggest you have treatment that is usually used to treat lymphoma. The treatments that you may have include the chemotherapy drug combinations of DHAP or ESHAP. We know from research that these sarcomas come back in the same place in about half of cases. This is called local recurrence. Spread to other parts of the body is seen in about 25 out of every 100 cases (25%) seen.
Coping with a diagnosis of cancer can be difficult, both practically and emotionally. It can be especially difficult if you have a rare cancer. Being well informed about your cancer and its treatment can make it easier to make decisions and cope with what happens.
It can also help to talk to other people who have the same thing. But it can be hard to find people who have had a rare type of cancer.
You can phone the Cancer Research UK nurses on freephone 0808 800 4040. The lines are open from 9am to 5pm, Monday to Friday. If you want to find people to share experiences with on line, you could use CancerChat, our online forum. It is a place for anyone affected by cancer to share experiences, stories and information with other people who know what you are going through. You are unlikely to find anyone with this particular type of sarcoma. But there are likely to be other people who’ve had similar treatment and can understand how the treatment affects you.
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