Follicular dendritic cell sarcoma (FDC sarcoma) is a very rare type of blood cancer. It is similar to lymphoma, but doctors usually treat it as a soft tissue sarcoma. FDC sarcoma develops from specialised cells in the lymph nodes. The lymph nodes are part of the lymphatic system, which helps to fight infection.
Most FDC sarcomas develop in the lymph nodes and are called nodal cancers. But about 3 out of 10 (30%) develop elsewhere in the body such as:
- head and neck area
- digestive system (bowel, stomach)
These FDC sarcomas are known as extranodal tumours, because they grow outside the lymph nodes.
We don’t know what causes follicular dendritic cell sarcoma. It's 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. This is a condition where non cancerous (benign) tumours develop in the lymph nodes. But we need more research to confirm this.
Researchers have also found that the Epstein Barr virus might 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, which means they grow slowly.
Other symptoms may include:
- a cough
- a sore throat
- difficulty in swallowing
- weight loss
You might have a number of tests. These include a:
- CT scan
- PET scan
- chest x-ray
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 proteins (markers) on the cells. Specific proteins include CD21, CD23 and CD35. Doctors can test for these proteins to confirm the diagnosis. The testing is called immunohistochemical staining.
As follicular dendritic cell sarcoma is very rare, doctors are still trying to work out the standard treatment. Your specialist will discuss this with you.
Your treatment depends on:
- how big the sarcoma is, and whether it has spread to other parts of the body
- how different the sarcoma cells are from normal cells and how fast they grow (grade)
- your general health and fitness
You might have surgery to remove the cancer, followed by chemotherapy or radiotherapy.
Coping with a diagnosis of a rare cancer can be especially difficult, both practically and emotionally. Being well informed about your sarcoma and its treatment can make it easier to make decisions and cope with what happens.
Sarcoma UK provides support and information for people affected by soft tissue and bone sarcoma.
The Rare Cancer Alliance offer support and information to people affected by rare cancers.
Talking to other people who have the same thing can also help.
Our discussion forum Cancer Chat is a place for anyone affected by cancer. You can share experiences, stories and information with other people who know what you are going through.