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Synovitis, Pigmented Villonodular

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Synovitis, Pigmented Villonodular

para>Uncommon in elderly patients пїЅ пїЅ

Initial Tests (lab, imaging)

Synovial fluid aspiration/arthrocentesis (2): пїЅ пїЅ

Primarily treated as outpatient with surgery пїЅ пїЅ

Close follow-up for recurrence and progression of disease пїЅ пїЅ

No restrictions пїЅ пїЅ

11 Murphey пїЅ пїЅMD, Rhee пїЅ пїЅJH, Lewis пїЅ пїЅRB, et al. Pigmented villonodular synovitis: radiologic-pathologic correlation. Radiographics. 2008;28(5):1493 " пїЅ1518.22 Tyler пїЅ пїЅWK, Vidal пїЅ пїЅAF, Williams пїЅ пїЅRJ, et al. Pigmented villonodular synovitis. J Am Acad Orthop Surg. 2006;14(6):376 " пїЅ385.33 Bruns пїЅ пїЅJ, Ewerbeck пїЅ пїЅV, Dominkus пїЅ пїЅM, 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 " пїЅ1053.44 Blay пїЅ пїЅJY, El Sayadi пїЅ пїЅH, Thiesse пїЅ пїЅP, et al. Complete response to imatinib in relapsing pigmented villonodular synovitis/tenosynovial giant cell tumor (PVNS/TGCT). Ann Oncol. 2008;19(4):821 " пїЅ822.55 Ravi пїЅ пїЅV, Wang пїЅ пїЅWL, Lewis пїЅ пїЅVO. Treatment of tenosynovial giant cell tumor and pigmented villonodular synovitis. Curr Opin Oncol. 2011;23(4):361 " пїЅ366.66 Cassier пїЅ пїЅPA, Gelderblom пїЅ пїЅH, Stacchiotti пїЅ пїЅS, 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 " пїЅ1655.

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 " пїЅ).
  • 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 synoviumMore rapidly destructive, with poorer prognosisCan encroach on neurovascular structuresContinued inflammation and joint erosions lead to articular cartilage destruction and subsequent osteoarthritis.LPVNSCharacterized by focal, pedunculated lesionsFavorable prognosis due to localized natureLow recurrence rate after surgery
  • DPVNS (more common than LPVNS)Characterized by involvement of most or all of the joint synoviumMore rapidly destructive, with poorer prognosisCan encroach on neurovascular structuresContinued inflammation and joint erosions lead to articular cartilage destruction and subsequent osteoarthritis.
  • LPVNSCharacterized by focal, pedunculated lesionsFavorable prognosis due to localized natureLow recurrence rate after surgery
  • 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.
  • Characterized by focal, pedunculated lesions
  • Favorable prognosis due to localized nature
  • Low recurrence rate after surgery
  • 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 jointsKnee/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
  • 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

  • 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 LPVNSDPVNSPoorly localized synovial thickening with varying degrees of periarticular bony erosionsLPVNSPeriarticular/synovial nodular mass with varying degree of bony erosionDPVNS and LPVNSProminent low-signal intensity seen on T1- and T2-weighted images secondary to high hemosiderin content
  • 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.
  • Underlying bone erosions/cysts with decreased signal
  • Modality of choice for diagnosis, determining the extent of the disease and differentiating DPVNS from LPVNS
  • DPVNSPoorly localized synovial thickening with varying degrees of periarticular bony erosions
  • LPVNSPeriarticular/synovial nodular mass with varying degree of bony erosion
  • DPVNS and LPVNSProminent low-signal intensity seen on T1- and T2-weighted images secondary to high hemosiderin content
  • Poorly localized synovial thickening with varying degrees of periarticular bony erosions
  • Periarticular/synovial nodular mass with varying degree of bony erosion
  • Prominent low-signal intensity seen on T1- and T2-weighted images secondary to high hemosiderin content
  • May demonstrate blood-tinged synovial fluid
  • Not pathognomonic sign of PVNS; lacks sensitivity and specificity

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

  • 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 treatmentComplete synovectomy by open surgical procedure for active diffuse PVNS.Arthroscopic partial synovectomy for local PVNSPatients 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 surgeryUseful in challenging cases with extensive extra-articular involvement, recurrent disease, or involvement of critical anatomic structuresSerious complications include skin reactions, poor wound healing, and sarcomatous transformation.Contraindicated in children and in those with prosthetic joints
  • 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.
  • 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

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

DIET

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.
  • Rates are higher with DPVNS.
  • Rates increase with extra-articular involvement.

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

ADDITIONAL READING

  • Al-Nakshabandi пїЅ пїЅNA, Ryan пїЅ пїЅAG, Choudur пїЅ пїЅH, et al. Pigmented villonodular synovitis. Clin Radiol. 2004;59(5):414 " пїЅ420.
  • de Carvalho пїЅ пїЅLHJr, Soares пїЅ пїЅLF, Gon пїЅ пїЅalves пїЅ пїЅMB, 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 " пїЅ1274.
  • Frassica пїЅ пїЅFJ, Bhimani пїЅ пїЅMA, McCarthy пїЅ пїЅEF, et al. Pigmented villonodular synovitis of the hip and knee. Am Fam Physician. 1999;60(5):1404 " пїЅ1410.
  • Verspoor пїЅ пїЅFG, van der Geest пїЅ пїЅIC, Vegt пїЅ пїЅE, et al. Pigmented villonodular synovitis: current concepts about diagnosis and management. Future Oncol. 2013;9(10):1515 " пїЅ1531.

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.