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Pigmented Villonodular Synovitis (PVNS)

A rare inflammatory granulomatous condition, of unknown aetiology, which causes proliferation of the synovium of joints , tendon sheaths or bursae. The disorder occurs most commonly in the third and fourth decades, but can occur at any age. It has no sex predilection and was first described by Jaffe et al. in 1941.

Pathological Features: 

  • Macroscopic Proliferation of synovial tissue can affect the whole of the synovium (diffuse), or a localised area (nodular). 

  • The synovium appears brown due to haemosiderin deposition within it. 

  • Microscopic: 

    • The hyperplastic synovium forms villi which are lined by synovial epithelial cells. 

    • Within the parenchyma there are large numbers of histiocytes, with other mesenchymal cells showing an increased number of mitoses. 

    • This condition was originally thought to be a low grade synovial malignancy, and is distinguished from a synovial sarcoma by the absence of histiocytes and haemosiderin.

Presentation: 

  • PVNS can affect any structure with a synovium. 

  • Intra articular PVNS tends to be of the diffuse form 

  • Tendon sheath PVNS the nodular form

  • The most common site is the knee, but other joints affected include hip, ankle, shoulder, elbow, wrist, TMJ, and rarely the spine. 

  • The patient will present with a monoarticular swelling with or without pain. 

  • Physical examination will reveal a soft tissue swelling, signs of synovial hypertrophy, and an effusion when the condition is chronic.

Diagnosis

  • Aspiration of the joint will characteristically reveal a blood tinged aspirate

  • Radiographic features are minimal in the early stages, with only soft tissue swelling visible. Later there will be increased density of the synovium, due to its haemosiderin content, and the surrounding bones may show cortical pressure erosions and subchondral cysts. Bone loss is a more common feature around the hip. 

  • MR scanning is the most useful, giving excellent delineation of the synovial disease, but it must be emphasised that MR cannot reliably distinguish between benign and malignant lesions

  • Synovial biopsy should be performed if there is any doubt



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