Diagnosis of pigmented villonodular synovitis
by Nathan Wei, MD, FACP, FACR
Nathan Wei is a nationally known board-certified rheumatologist and author of the Second Opinion Arthritis Treatment Kit. It's available exclusively at this website... not available in stores.
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Pigmented villonodular synovitis (PVNS) is a benign, locally invasive tumor of the joint capsule.
While the most common location is the knee, it can be found in other joints such as the hip, ankle, and elbow.
PVNS usually presents with acute attacks of pain and swelling. Diagnostic needle aspiration of the joint yields fluid that is either bloody or dark brown in color. The major other diagnostic consideration is rheumatoid arthritis.
The most common presentation of PVNS is a joint with a bloody effusion. There are two forms of PVNS... a nodular form or a diffuse form. Joint erosions can be seen on x-ray. The nodular form is not as common nor is it as destructive as the diffuse form of PVNS. Recurrent bloody effusions or recurrent effusions with normal color can be seen.
MRI is diagnostic; however, other diseases can mimic the appearance of PVNS. These include: hemophilic arthropathy, soft-tissue sarcoma, fibromatosis, synovial chondromatosis, septic arthritis, and other forms of inflammatory arthritis.
Pathologically, PVNS shows spindle cells with prominent cytoplasm and pale nuclei in the subsynovial space. Histiocyte-like cells are also present as are lipid-filled foam cells and multinucleated giant cells that mixed in with hemosiderin-laden cells.
Treatment of the nodular form is usually arthroscopic synovectomy followed by low dose radiation therapy. Open synovectomy is the treatment advocated by some for diffuse disease.
The recurrence rate is high and therefore extensive synovectomy needs to be performed. Total knee replacement is indicated for PVNS with joint destructive changes.
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