para>Uncommon in elderly patients � �
Pediatric Considerations
Uncommon in children
� �
ETIOLOGY AND PATHOPHYSIOLOGY
- Exact cause is unknown.
- History of trauma is noted in ~50% of patients with DPVNS of the knee.
- DPVNS is thought to be either an inflammatory synovial reaction or a benign neoplastic process.
- Histologically, LPVNS and DPVNS are similar.
- Characterized by mononuclear stromal cells infiltrating synovial membrane
- Hemosiderin-loaded macrophages give the typical brown color ( "pigmented " �).
- Multinucleated giant cells and foam cells are also involved.
- DPVNS and LPVNS differ in their disease course.
- DPVNS (more common than LPVNS)
- Characterized by involvement of most or all of the joint synovium
- More rapidly destructive, with poorer prognosis
- Can encroach on neurovascular structures
- Continued inflammation and joint erosions lead to articular cartilage destruction and subsequent osteoarthritis.
- LPVNS
- Characterized by focal, pedunculated lesions
- Favorable prognosis due to localized nature
- Low recurrence rate after surgery
Genetics
- Translocation of collagen 6A3 gene (2q35) and macrophage colony-stimulating factor gene (1p13) in a fraction of tumor cells
- Cytogenetic aberrations are seen in most cases.
- Overexpression of colony-stimulating factor 1
DIAGNOSIS
HISTORY
- Typically, monarticular involving large joints
- Knee/hip are most commonly involved joints.
- Ankle, shoulder, elbow, and spine are less common.
- Onset of symptoms can be insidious or acute and episodic.
- Pain, swelling, and stiffness are common in DPVNS.
- History of locking, catching, or instability is common with LPVNS.
- Patients occasionally report periods of excessive pain, which may correspond to hemorrhage into the joint space.
- Symptoms often intermittent and poorly localized
PHYSICAL EXAM
- May be completely normal
- May have tenderness to palpation of involved joint such as on patellofemoral area in knee DPVNS
- May have moderate-to-large joint effusion
- May have stiffness with decreased active and passive range of motion of involved joint
DIFFERENTIAL DIAGNOSIS
- Rheumatoid arthritis; osteoarthritis; psoriatic arthritis
- Septic arthritis
- Inflammatory arthritis
- Synovial sarcoma
- Avascular necrosis
- Systemic lupus erythematosus (SLE)
- Gout
- Other benign/malignant bone tumor
DIAGNOSTIC TESTS & INTERPRETATION
Initial Tests (lab, imaging)
- Erythrocyte sedimentation rate (ESR)
- C-reactive protein (CRP)
- Antinuclear antibody (ANA)
- Rheumatoid factor (RF)
- CBC
- Uric acid
- Joint fluid analysis
- Plain radiographs (1,2)
- Most cases have normal radiographs.
- Findings seen more commonly in hip joint than in knee joint because the knee capsule can stretch and hold the proliferating hyperplastic synovium.
- Findings include bony erosions with thin rim of sclerotic margins.
- Joint space narrowing is a late finding.
- CT scan (1,2)
- Underlying bone erosions/cysts with decreased signal
- MRI (1,2)
- Modality of choice for diagnosis, determining the extent of the disease and differentiating DPVNS from LPVNS
- DPVNS
- Poorly localized synovial thickening with varying degrees of periarticular bony erosions
- LPVNS
- Periarticular/synovial nodular mass with varying degree of bony erosion
- DPVNS and LPVNS
- Prominent low-signal intensity seen on T1- and T2-weighted images secondary to high hemosiderin content
Diagnostic Procedures/Other
Synovial fluid aspiration/arthrocentesis (2): � �
- May demonstrate blood-tinged synovial fluid
- Not pathognomonic sign of PVNS; lacks sensitivity and specificity
Test Interpretation
See " Etiology and Pathophysiology. " � � �
TREATMENT
GENERAL MEASURES
- Treatment of PVNS is often surgical (2)[A].
- Synovectomy removes abnormal synovium and is the treatment of choice for, reducing the risk of joint destruction and recurrence.
- Use of adjuvant treatments along with surgery is recommended, particularly for diffuse PVNS, to prevent recurrence (3)[C].
ADDITIONAL THERAPIES
Pharmacotherapy (4,5)[C] � �
- TNF-α (tumor necrosis factor-α) blockade: after surgical excision of abnormal synovium
- Infliximab, sunitinib, and imatinib may inhibit CSF1R activation (5)[C],(6).
- May also induce complete response and be used as an option for nonsurgical treatment; must balance symptom alleviation with drug toxicities (4)[C]
- Bisphosphonates may inhibit periarticular bone destruction carried out by osteoclasts in PVNS.
SURGERY/OTHER PROCEDURES
- Surgical resection (2,5)[C]
- Mainstay of treatment
- Complete synovectomy by open surgical procedure for active diffuse PVNS.
- Arthroscopic partial synovectomy for local PVNS
- Patients with large popliteal masses or extra-articular involvement are generally not candidates for arthroscopic surgery.
- Arthroscopic treatment of DPVNS is associated with higher recurrence rate, unlike rare recurrence after limited local treatment in LPVNS.
- Joint replacement is indicated in patients with significant joint destruction.
- Radiation therapy (1),(3)[C]
- Recommended as an adjuvant to surgery
- Useful in challenging cases with extensive extra-articular involvement, recurrent disease, or involvement of critical anatomic structures
- Serious complications include skin reactions, poor wound healing, and sarcomatous transformation.
- Contraindicated in children and in those with prosthetic joints
INPATIENT CONSIDERATIONS
Primarily treated as outpatient with surgery � �
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
Close follow-up for recurrence and progression of disease � �
DIET
No restrictions � �
PROGNOSIS
- Patients with recurrent disease have more extensive involvement and less chance for successful treatment.
- DPVNS is more rapidly destructive and has a poorer prognosis.
- Recurrence rates after synovectomy range from 8% to 20%.
- Rates are higher with DPVNS.
- Rates increase with extra-articular involvement.
- Articular cartilage destruction and the development of osteoarthritis may hasten joint replacement surgery.
- Malignant transformation is rare.
COMPLICATIONS
- Surrounding cortical erosion and tissue damage resulting in limited ambulation and debilitating pain
- Extensive joint involvement and extra-articular spread may result after failed arthroscopic management.
- Recurrence may lead to joint replacement.
- Postoperative stiffness occurs in ~25% of patients after open procedure.
REFERENCES
11 Murphey � �MD, Rhee � �JH, Lewis � �RB, et al. Pigmented villonodular synovitis: radiologic-pathologic correlation. Radiographics. 2008;28(5):1493 " �1518.22 Tyler � �WK, Vidal � �AF, Williams � �RJ, et al. Pigmented villonodular synovitis. J Am Acad Orthop Surg. 2006;14(6):376 " �385.33 Bruns � �J, Ewerbeck � �V, Dominkus � �M, et al. Pigmented villo-nodular synovitis and giant-cell tumor of tendon sheaths: a binational retrospective study. Arch Orthop Trauma Surg. 2013;133(8):1047 " �1053.44 Blay � �JY, El Sayadi � �H, Thiesse � �P, et al. Complete response to imatinib in relapsing pigmented villonodular synovitis/tenosynovial giant cell tumor (PVNS/TGCT). Ann Oncol. 2008;19(4):821 " �822.55 Ravi � �V, Wang � �WL, Lewis � �VO. Treatment of tenosynovial giant cell tumor and pigmented villonodular synovitis. Curr Opin Oncol. 2011;23(4):361 " �366.66 Cassier � �PA, Gelderblom � �H, Stacchiotti � �S, et al. Efficacy of imatinib mesylate for the treatment of locally advanced and/or metastatic tenosynovial giant cell tumor/pigmented villonodular synovitis. Cancer. 2012;118(6):1649 " �1655.
ADDITIONAL READING
- Al-Nakshabandi � �NA, Ryan � �AG, Choudur � �H, et al. Pigmented villonodular synovitis. Clin Radiol. 2004;59(5):414 " �420.
- de Carvalho � �LHJr, Soares � �LF, Gon � �alves � �MB, et al. Long-term success in the treatment of diffuse pigmented villonodular synovitis of the knee with subtotal synovectomy and radiotherapy. Arthroscopy. 2012;28(9):1271 " �1274.
- Frassica � �FJ, Bhimani � �MA, McCarthy � �EF, et al. Pigmented villonodular synovitis of the hip and knee. Am Fam Physician. 1999;60(5):1404 " �1410.
- Verspoor � �FG, van der Geest � �IC, Vegt � �E, et al. Pigmented villonodular synovitis: current concepts about diagnosis and management. Future Oncol. 2013;9(10):1515 " �1531.
CODES
ICD10
- M12.20 Villonodular synovitis (pigmented), unspecified site
- M12.259 Villonodular synovitis (pigmented), unspecified hip
- M12.269 Villonodular synovitis (pigmented), unspecified knee
- M12.279 Villonodular synovitis (pigmented), unsp ankle and foot
- M12.261 Villonodular synovitis (pigmented), right knee
- M12.252 Villonodular synovitis (pigmented), left hip
- M12.251 Villonodular synovitis (pigmented), right hip
- M12.262 Villonodular synovitis (pigmented), left knee
- M12.271 Villonodular synovitis (pigmented), right ankle and foot
- M12.29 Villonodular synovitis (pigmented), multiple sites
- M12.249 Villonodular synovitis (pigmented), unspecified hand
- M12.28 Villonodular synovitis (pigmented), vertebrae
- M12.221 Villonodular synovitis (pigmented), right elbow
- M12.242 Villonodular synovitis (pigmented), left hand
- M12.211 Villonodular synovitis (pigmented), right shoulder
- M12.219 Villonodular synovitis (pigmented), unspecified shoulder
- M12.272 Villonodular synovitis (pigmented), left ankle and foot
- M12.222 Villonodular synovitis (pigmented), left elbow
- M12.229 Villonodular synovitis (pigmented), unspecified elbow
- M12.231 Villonodular synovitis (pigmented), right wrist
- M12.232 Villonodular synovitis (pigmented), left wrist
- M12.239 Villonodular synovitis (pigmented), unspecified wrist
- M12.241 Villonodular synovitis (pigmented), right hand
- M12.212 Villonodular synovitis (pigmented), left shoulder
ICD9
- 719.20 Villonodular synovitis, site unspecified
- 719.25 Villonodular synovitis, pelvic region and thigh
- 719.26 Villonodular synovitis, lower leg
- 719.27 Villonodular synovitis, ankle and foot
- 719.29 Villonodular synovitis, multiple sites
- 719.28 Villonodular synovitis, other specified sites
- 719.23 Villonodular synovitis, forearm
- 719.22 Villonodular synovitis, upper arm
- 719.21 Villonodular synovitis, shoulder region
- 719.24 Villonodular synovitis, hand
SNOMED
- Pigmented villonodular synovitis
- Pigmented villonodular synovitis of hip joint
- Pigmented villonodular synovitis of knee joint
- Villonodular synovitis of the ankle and foot (disorder)
- Villonodular synovitis of the pelvic region and thigh (disorder)
- Villonodular synovitis of upper limb (disorder)
CLINICAL PEARLS
- PVNS is characterized by synovial inflammation and hemosiderin deposition. The etiology is unknown.
- Most frequent in 3rd and 4th decades of life
- Typically presents insidiously over time with intermittent episodes of joint pain and swelling. The knee is the most commonly involved joint.
- MRI is the diagnostic modality of choice for PVNS.
- Surgery is mainstay of treatment. Pharmacotherapy and radiotherapy can be used as adjuncts.