Pigmented Villonodular synovitis

- Discussion:
    - pigmented villonodular synovitis is a slow growing, benign, and locally invasive tumor of the synovium;
    - location: most often involves the knee (also in hip, ankle, elbow, etc.);
            - always consider PVNS in a younger patient with unexplained hip pain;
    - acute episodic attacks of pain and swelling may occur;
    - patients may have mechanical symptoms (locking and catching);
    - most have hemorrhagic, dark brown synovial fluid;
    - biopsy is diagnostic;
    - diff dx: rheumatoid arthritis: unlike PVNS, RA tends to affect multiple joints;
    - sub-types:
            - it usually presents as a monoarticular hemarthrosis, and may exist in a nodular or a diffuse form;
            - diffuse form:
                   - disease may be active or inactive;
                   - look for peri-articular erosions on radiographs;
                   - diffuse mass may be present on exam;
            - nodular form:
                   - less common than the diffuse form of the disease;
                   - does not show the same destructive changes as the diffuse form of PVNS;
                   - may cause recurrent hemarthrosis and aspirate may be of normal color (may not show classic brown color);

- Radiographs:
    - reference: Roentgenographic findings in pigmented villonodular synovitis of the knee.

- MRI Findings:
    - most characteristic feature of PVNS on MRI is the presence of intraarticular nodular masses of low signal intensity on T1,
             T2-weighted, and proton-density-weighted images;
    - diseased synovium and focal masses may be best demonstrated on T2 images, showing up as a void of signal intensity felt due to hemosiderin deposition;
    - hypo-intense signal is also seen on T1 images;
    - MRI differential diagnosis:
           - hemophilic arthropathy
           - soft-tissue sarcoma
           - fibromatosis
           - synovial chondromatosis
           - septic arthritis
           - inflammatory arthropathies
           - hemorrhagic synovitis
    - references:
           - Magnetic resonance imaging in the diagnosis of aggressive villonodular synovitis.
           - The use of magnetic resonance imaging in the diagnosis of pigmented villonodular synovitis.
           - The use of MRI to assist in diagnosis of pigmented villonodular synovitis of the knee joint.
           - Magnetic resonance imaging evaluation and arthroscopic resection of localized pigmented villonodular synovitis of the knee.                   

- Histology:
    - histologic characteristics are similar to GCT of tendon sheath;
    - classic cytoarchitecture consists of subsynovial nodular proliferation of large round, polyhedral, or spindle cells with prominent cytoplasm and pale nuclei;
    - phagocytic histiocyte-like cells are also present;
    - lipid-laden foam cells and multinucleated giant cells are interspersed with hemosiderin-laden cells that resemble lining cells;
    - giant cells appear to have phagocytic role;
    - findings include deposition of iron, foam cells, or giant cells;

- Treatment:
    - arthroscopic synovectomy:
          - may be indicated for nodular form or for inactive form of diffuse disease;
          - some authors stress the importance of a synovectomy of the posterior compartment;
          - references:
                 - Pigmented villonodular synovitis of the knee. The results of total arthroscopic synovectomy, partial, arthroscopic synovectomy, and arthroscopic local excision.  
                 - Does Combined Open and Arthroscopic Synovectomy for Diffuse PVNS of the Knee Improve Recurrence Rates? 
                 - Localized pigmented villonodular synovitis of the knee. Arthroscopic treatment.
- Result of arthroscopic treatment of pigmented villonodular synovitis of the knee.
    - radiation therapy:

          - Blanco, et al: results of combined partial arthroscopic synovectomy and low-dose xrt in the treatment of diffuse PVNS of the knee;
                 - PRS of 22 patients with clinical, ultrasonic, and histologically confirmed findings of diffuse PVNS of the knee;
                 - protocol included anterior (patellofemoral, medial, and lateral) arthroscopic synovectomy and postop RT with a total dose of 2,600 cGy;
                 - combination therapy was effective in reducing symptoms of pain and edema, and in improving overall function of patients;
                 - 19 patients (86%) had good or excellent results at an average follow-up of 33 months (range, 26 to 76 months);
                 - 3 had confirmed recurrence of disease and were treated with repeat arthroscopic synovectomy w/o harmful effects from RT;
          - references:
                 - Combined partial arthroscopic synovectomy and radiation therapy for diffuse pigmented villonodular synovitis of the knee
                 - The effect of surgical synovectomy and radiotherapy on the rate of recurrence of pigmented villonodular synovitis of the knee: an individual patient meta-analysis.
    - open synovectomy:
          - treatment of choice for patients w/ active form of diffuse disease;
          - recurrence rate is relatively high and therefore both anterior and posterior open synovectomy is required;
    - total knee arthroplasty:
          - indicated for PVNS with joint destructive changes;
          - posterior stabilized prosthesis is recommended in order to maximize the exposure as well as the open synovectomy;
          - in the report by Hamlin BR, et al (1997), out of 13 patients w/ active diffuse PVNS who underwent TKR, 2 had recurrence
          - ref: Total knee arthroplasty in patients who have pigmented villonodular synovitis.

Pigmented villonodular synovitis of the wrist and adjacent bones.

Pigmented villonodular synovitis. A retrospective review of affected large joints.

Pigmented villonodular synovitis (giant-cell tumor of the tendon sheath and synovial membrane). A review of eighty-one cases.

Surgical treatment of diffuse pigmented villonodular synovitis of the knee.

Pigmented villonodular synovitis

Pigmented villonodular synovitis of synovial joints: clinical, pathologic, and radiologic features.

Pigmented Villonodular Synovitis: Keys to Early Diagnosis.

Treatment of Advanced Primary and Recurrent Diffuse Pigmented Villonodular Synovitis of the Knee.

Localized pigmented villonodular synovitis of the knee.

Pigmented villonodular synovitis about the ankle: a review of the literature and presentation in 10 athletic patients.

Surgical Treatment of Pigmented Villonodular Synovitis of the Hip.

What Affects the Recurrence and Clinical Outcome of Pigmented Villonodular Synovitis?

Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Friday, July 10, 2015 5:14 am