Pigmented villonodular synovitis

What causes pigmented villonodular synovitis?

The exact cause of pigmented villonodular synovitis (PVNS) 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. The association between a history of trauma and the development of PVNS is unclear. One study found that 56% of individuals with PVNS had a history of previous trauma, while other studies have found a much lower incidence.

There have been studies suggesting that PVNS could be caused by specific genetic changes in the cells lining the joint. More studies are needed to research this association.

Last updated on 05-01-20

How is pigmented villonodular synovitis diagnosed?

Pigmented villonodular synovitis (PVNS) is diagnosed via physician examination, imaging studies, and sometimes surgical procedures. Imaging studies commonly used include: X-ray, MRI, and CT scan. MRI findings are diagnostic in more than 95% of patients. CT scan findings are additionally often diagnostic, though they might not show the extent of the disease.

Other methods that might be utilized in the diagnostic process include joint aspiration, in which a needle is used to remove fluid from the joint and a biopsy, in which a small operation is completed to obtain a tissue sample.

Last updated on 05-01-20

How common is pigmented villonodular synovitis?

Pigmented villonodular synovitis (PVNS) is considered rare. Estimates of prevalence may vary depending on the type of PVNS (localized or diffuse). According to a recently published study based on registry data in Denmark, the prevalence in 2012 was 44 per 100,000 people for localized PVNS, and 11 per 100,000 people for diffuse PVNS. This means that PVNS may occur in about 5-6 people out of 10,000 people. We are not aware of recent prevalence estimates of PVNS in the United States.

Last updated on 05-01-20

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.

Last updated on 05-01-20

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Drug Name Generic Name
Turalio pexidartinib

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