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Genetic and Rare Diseases Information Center (GARD)

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Pigmented villonodular synovitis

Other Names for this Disease
  • Diffuse pigmented villonodular synovitis
  • Localized pigmented villonodular synovitis
  • Tenosynovial giant cell tumors
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Your Question

I have just received a diagnosis of pigmented villonodular synovitis. Can you tell me more about this condition?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is pigmented villonodular synovitis?

Pigmented villonodular synovitis is a disease in which the tissue lining a joint in the body begins to grow abnormally.  There are two types of pigmented villonodular synovitis: the nodular form (where the abnormal tissue growth forms a single bump) and the diffuse form (where the entire lining of the joint grows unusually).  The additional tissue in the joint can cause pain, limit movements or cause the joint to lock, and in some cases, can destroy the normal joint structure.  The knee is most commonly affected by this condition, though it can occur in other joints such as the hip, shoulder, elbow, ankle, or wrist.  The average age of diagnosis for this condition is 35 years.[1]
Last updated: 11/28/2011

What causes pigmented villonodular synovitis?

The exact cause of pigmented villonodular synovitis is unknown.  Some doctors believe that it is similar to arthritis, arising from swelling (inflammation) of the joint tissue.  Others believe it develops like a tumor, caused by cells growing and multiplying more quickly than usual.  [1]  A recent study suggests that pigmented villonodular synovitis could be caused by specific genetic changes in the cells lining the joint, which supports the theory that this condition is a type of tumor.[2]
Last updated: 11/28/2011

How might pigmented villonodular synovitis be treated?

Pigmented villonodular synovitis is first treated with surgery to remove as much of the abnormal tissue growth as possible.  The type of surgery depends on the location and extent of the disease within the joint.  Radiation therapy is sometimes used to treat this condition if surgery is not an option, or if the condition returns (recurs) after an initial surgery.[1]  More recently, research is being done on the use of certain medications (systemic therapy), such as imatinib and sunitinib, to treat this condition.[2]
Last updated: 11/28/2011

How common is pigmented villonodular synovitis?

Pigmented villonodular synovitis is rare, affecting two in a million people.[1]
Last updated: 11/28/2011

  • Mendenhall WM, Mendenhall CM, Reith JD, Scarborough MT, Gibbs CP, Mendenhall NP. Pigmented Villonodular Synovitis. American Journal of Clinical Oncology. 2006; 29:548-550. Accessed 11/22/2011.
  • Ravi V, Wang WL, Lewis VO. Treatment of tenosynovial giant cell tumor and pigmented villonodular synovitis. Current Opinion in Oncology. 2011; 23:361-366. Accessed 11/22/2011.