Updated: 5/31/2021

Pigmented Villonodular Synovitis

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  • Summary
    • Pigmented Villonodular Synovitis (PVNS) is a locally aggressive neoplastic synovial disease (not a true neoplasm) characterized by joint effusions, expansion of the synovium, and bony erosions.
    • Diagnosis is multifaceted with clinical assessment for joint effusion (most commonly the knee), MRI studies showing synovial expansion, arthrocenesis revealing a brown fluid, and biopsy revealing hemosiderin-stained multinucleated giant cells.
    • Treatment generally consists of partial or total surgical synovectomy depending on presence of localized or diffuse PVNS. 
  • Epidemiology
    • Incidence
      • rare
        • 9.2 per million per year in the United States
    • Demographics
      • most commonly in adults age 30-40 but can occur at any age
      • equal incidence in men and women
    • Anatomic location
      • may occur locally (within a joint) or diffusely
      • localized (intra-articular or classic form)
        • anterior knee is the most common site of involvement (80%)
          • most commonly affects the patellofemoral compartment at the infrapatellar fat pad
        • knee > hip > ankle > shoulder > elbow
      • diffuse (extra-articular extension)
        • behaves differently from localized
  • Etiology
    • Pathophysiology
      • pathobiology
        • caused by an overexpression of CSF1 gene
          • overexpression leads to clusters of aberrant cells creating focal areas of soft tissue hyperplasia in the synovial cells lining joints
        • a locally aggressive neoplastic synovial disease (not a true neoplasm)
    • Genetics
      • mutations
        • locations of chromosome 1p13 in majority of cases
        • 5q33 chromosomal rearrangement
    • Associated conditions
      • Giant Cell Tumor of Tendon Sheath
        • also known as pigmented villonodular tumor of the tendon sheath (PVNTS)
  • Classification
    • Localized versus Diffuse PVNS
      Characteristic
      Localized PVNS
      Diffuse PVNS
      Location
      Knee > hip > ankle
      Knee (75%)
      Age
      30-50y
      <40y
      Gender
      Male = Female
      Female =/> Male
      Presentation

      Painless, swollen joint, longstanding
      Painful, swollen, tender, limited mobility
      Radiograph

      Osseous erosion from localized pressure
      Degenerative changes on both sides of the joint
      MRI

      Well circumscribed soft tissue mass
      Ill-defined (poorly circumscribed) soft tissue mass
      Recurrence
      8% after synovectomy
      30% after synovectomy
  • Presentation
    • History
      • 50% of patients will have a prior history of trauma to the area
    • Symptoms
      • common symptoms
        • insidious onsent of pain in affected joint
        • stiffness in the affected joint
        • swelling in the affected joint
      • recurrent atraumatic hemarthrosis
        • is hallmark of disorder
    • Physical exam
      • inspection
        • joint effusion
        • erythema
      • palpation
        • tenderness along joint line
      • motion
        • limited motion of affected joint
  • Imaging
    • Radiographs
      • recommended views
        • AP and lateral of affected joint
      • findings
        • soft tissue swelling
        • may show cystic erosion with sclerotic margins on both sides of the joint
    • CT
      • indications
        • to evaluate for extent of cystic bone loss
      • findings
        • may show cystic erosions on both sides of the joint similar to radiographs
    • MRI
      • indications
        • most sensitive imaging study
        • provides excellent delineation of both intra-articular and extra-articular disease
      • findings
        • reveals joint effusion, hemosiderin deposits, expansion of the synovium, and bony erosion
        • low signal intensity on T1
          • due to hemosiderin deposits
          • presence of fat signal (T1) within the lesion
        • low signal intensity on T2
        • "blooming artifact"
          • signal loss on gradient-echo sequences
          • because of iron in hemosiderin
        • extra-articular extension
          • commonly see posterior extension outside of the knee joint of an intra-articular process
  • Studies
    • Labs
      • CRP and ESR
        • often normal despite signs of soft tissue swelling
    • Arthrocentesis
      • indication
        • recurrent hemarthrosis
      • findings
        • grossly bloody effusion
    • Diagnostic arthroscopy
      • indications
        • gold standard for diagnosis
        • synovial biopsy should be performed
      • findings
        • brownish or reddish inflamed synovium is typical of PVNS
        • frond-like pattern of papillary projections
    • Histology
      • gross histology
        • shows a proliferative mass extending from the synovium
      • low power
        • mononuclear stromal cells infiltrating the synovium
        • highly vascular villi lined with plump hyperplastic synovial cells
      • high power
        • hemosiderin stained multinucleated giant cells
        • pigmented foam cells (lipid-laden histiocytes)
        • mitotic figures common
  • Differential
    • Synovial chondromatosis
    • Hemophilia/hemarthrosis
    • Rheumatoid arthritis
    • Septic joints
    • Other neoplasia
  • Treatment
    • Nonoperative
      • observation
        • indications
          • asymptomatic disease only
      • CSF-1 receptor antagonist (pexidartinib)
        • indications
          • approved in 2019 for use in patients with extensive disease who are not likely to benefit from surgical intervention
    • Operative
      • partial synovectomy
        • indications
          • local form of PVNS
        • technique
          • if lesion is accessible from anterior knee, this is can be done arthroscopically
          • posterior or extra-articular lesions should be performed open
      • total synovectomy +/- external beam radiation
        • indications
          • in grossly symptomatic and painful disease
        • technique
          • total synovectomy is classified as marginal excision
          • techniques range from arthroscopic to fully open total synovectomy depending on extent and location of disease
        • outcomes
          • improved functional and range of motion outcomes with arthroscopic technique
          • frequent recurrence is common
            • mostly due to incomplete synovectomy
        • external beam irradiation
          • technique
            • 30-35Gy in 15 fractions, or 50Gy in 25 fractions
          • outcomes
            • when combined with total synovectomy, reduces rate of recurrence to 10-20%
      • total synovectomy and total joint arthroplasty
        • indications
          • advanced disease with severe degenerative joint changes i knee, hip, and shoulder
      • total synovectomy and arthrodesis
        • indications
          • severe disease of the ankle
  • Techniques
    • CSF-1 receptor antagonist (pexidartinib)
      • technique
        • oral medication taken once daily for 24 weeks showed significant improvement of PVNS disease burden in ~40% of patients.
      • complications
        • cholestatic hepatotoxicity was a noted side-effect of the drug
    • Arthroscopic synovectomy of knee for PVNS
      • approach
        • routine arthroscopic portals for knee, ankle, and shoulder
      • technique
        • perform as thorough resection of synovium as possible through portals
        • can be challenging to access the posterior portions of the joint or extra-articular disease
    • Open posterior synovectomy of knee for PVNS
      • approach
        • posterior approach to the knee via transverse or S-shape incision across popliteal fossa
        • approach between medial and lateral heads of gastrocnemius
        • retract neurovascular bundle to access posterior joint capsule
      • technique
        • disease is often seen posterior and extra-articular to the knee
        • complete posterior synovectomy and resection of extra-articular disease
      • complications
        • posterior approach to the knee places popliteal neurovascular bundle at risk
  • Complications
    • Recurrence
      • incidence
        • recurrence is the most frequent complication for both intra-articular and extra-articular disease
          • 30%-50% recurrence rate despite complete synovectomy
            • same rates for complete open vs open+arthroscopic
          • rates can be reduced with addition of external beam radiation
    • Joint destruction
      • moderate to severe joint deformity
      • treatment
        • may lead to the need for arthrodesis or amputation
    • Skin necrosis, radiation induced sarcoma
      • risk factors
        • radiation therapy
  • Prognosis
    • PVNS is associated with a high rate of recurrence and accelerated degenerative changes of the knee ultimately requiring arthroplasty
      • TKA in patients with PVNS is associated with complication rates
Questions (19)
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(SBQ12FA.102) Figures A is the MRI image of a 31-year-old female who presents with complaints of worsening ankle pain and limited motion for the past 10 months. She denies any trauma to the ankle. She ambulates without issue but admits to recurrent swelling and redness of the ankle. She is afebrile and CBC, ESR, and CRP are unremarkable. She underwent an aspiration which revealed 1,100 WBCs with 40% PMNs and >50,000 RBCs. She undergoes a biopsy of the lesion and the histologic finding is seen in Figure B. Which is the most appropriate next step in management?

QID: 3909
FIGURES:
1

Arthroscopic or open resection of the lesion

75%

(989/1319)

2

Chemotherapy with wide resection of the lesion

11%

(151/1319)

3

External beam radiation followed by radical resection of the lesion

10%

(128/1319)

4

Observation with serial radiographs

2%

(20/1319)

5

Physical therapy with a repeat MRI in 6 months

2%

(26/1319)

L 3 B

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(OBQ12.122) A 38-year-old male presents with right knee pain that is progressively worsening over the past 5 months. He is unable to perform his occupation as a construction worker and had to stop playing tennis. He denies constitutional symptoms. The patient had an effusion that was drained by his primary care provider 2 months ago and he took a picture of it on his cell phone to show you (Figure A). On examination there is an effusion in the knee and there is tenderness along the parapatellar region. He has full knee extension and flexion, but open chain knee extension exacerbates his pain. His Lachman examination demonstrates 3 mm of anterior translation with an endpoint noted. A radiograph is shown in Figure B and MRI images are shown in Figure C and D. What is the next most appropriate step in management?

QID: 4482
FIGURES:
1

Neoadjuvant chemotherapy, surgical resection, adjuvant chemotherapy

4%

(190/4888)

2

Neoadjuvant external beam radiation, wide surgical resection, and adjuvant chemotherapy

4%

(172/4888)

3

Arthroscopic or open resection of the lesion

86%

(4217/4888)

4

Observation with serial radiographs and physical therapy for lower leg and core strengthening

3%

(127/4888)

5

Arthroscopic or open plica removal and lateral retinacular release

3%

(138/4888)

L 2 B

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(OBQ09.221) A 24-year-old man presents with pain and a mass in the anterior ankle. Plain radiographs are normal, MRI is shown in Figure A (arrrows indicate the mass), and biopsy is shown in Figure B. What is the most likely diagnosis?

QID: 3034
FIGURES:
1

Aneurysmal bone cyst

1%

(32/2847)

2

Periosteal osteosarcoma

2%

(45/2847)

3

Chondroblastoma

2%

(65/2847)

4

Myositis ossificans

1%

(27/2847)

5

Pigmented villonodular synovitis (PVNS)

94%

(2664/2847)

L 1 B

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(OBQ08.31) Intralesional surgical resection is considered standard of care for which of the following conditions?

QID: 417
1

High grade sarcoma surrounding a major nerve

6%

(120/2015)

2

Intermediate grade solitary fibrous tumor

12%

(244/2015)

3

Atypical lipomatous tumor

10%

(204/2015)

4

Tibial adamantinoma

7%

(139/2015)

5

Pigmented villonodular synovitis

65%

(1300/2015)

L 2 C

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(SAE07SM.5) A 31-year-old woman has increasing pain and tightness in her right knee, with occasional stiffness and recurrent hemorrhagic effusions. MRI scans are shown in Figures 2a and 2b. What is the most likely diagnosis?

QID: 8667
FIGURES:
1

Rheumatoid arthritis

0%

(3/603)

2

Pigmented villonodular synovitis (PVNS)

89%

(534/603)

3

Synovial sarcoma

4%

(26/603)

4

Synovial chondromatosis

5%

(33/603)

5

Fibromatosis

0%

(1/603)

L 2 E

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(SBQ07SM.5) A 45-year-old male presents with a 6-month history of recurrent knee effusions and pain. There is no history of trauma. Exam shows a large joint effusion, decreased range of motion, and a nodularity surrounding the knee. MRI and arthroscopic intra-articular picture are shown. What is the most likely diagnosis?

QID: 1390
FIGURES:
1

Synovial sarcoma

2%

(29/1655)

2

Rheumatoid nodule

0%

(5/1655)

3

Septic arthritis

0%

(6/1655)

4

Synovial chondromatosis

4%

(67/1655)

5

Pigmented villonodular synovitis

93%

(1536/1655)

L 1 C

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