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Follicular dendritic cell sarcoma

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)
  • spleen
  • liver

These FDC sarcomas are known as extranodal tumours, because they grow outside the lymph nodes.

Causes

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.

Symptoms

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
  • tiredness

Getting diagnosed

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.

Protein markers

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.

Treatment

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

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.

Last reviewed: 
09 Jul 2018
  • Follicular dendritic cell sarcoma: two rare cases and a brief review of the literature

    Y Ma and others

    Oncotargets and therapy, 2015 .Volume 8, pages 1823-1830

  • Distinctive Histogenesis and Immunological Microenvironment Based on Transcriptional Profiles of Follicular Dendritic Cell Sarcomas

    M Laginestra and others

    Molecular Cancer Research 2017 DOI: 10.1158/1541-7786.MCR-16-0301

  • miRNA expression profiling divides follicular dendritic cell sarcomas into two groups, related to fibroblasts and myopericytomas or Castleman’s disease

    S Hartmann and others

    European Journal of Cancer, 2016. Volume 64, pages 159-166

  • Impact of surgery, radiation and systemic therapy on the outcomes of patients with dendritic cell and histiocytic sarcomas

    M Gounder and others

    European Journal of Cancer, 2016. Volume 51, pages 2413-2422

  • Next-generation sequencing of idiopathic multicentric and unicentric Castleman disease and follicular dendritic cell sarcomas

    A Nagy and others

    Blood Advances, 2018. Volume 2, number 5, pages 481-491

  • The information on this page is based on literature searches and specialist checking. We used many references and there are too many to list here. Please contact patientinformation@cancer.org.uk with details of the particular issue you are interested in if you need additional references for this information.

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