Updated: 12/10/2019

Pigmented Villonodular Synovitis

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Introduction
  • PVNS is an idiopathic monoarticular neoplastic synovial disease 
    • characterized by exuberant proliferation of synovial villi and nodules
  • Epidemiology
    • demographics
      • most commonly in adults age 30-40 but can occur at any age 
        • prior test question patient ages have been 16, 30, 31, 37 years
      • equal incidence in men and women
    • types
      • localized (intra-articular or classic form) 
        • knee is the most common site of involvement (80%)
        • knee > hip > ankle > shoulder > elbow
      • diffuse (extra-articular extension)
        • behaves differently from localized
      • giant cell tumor of tendon sheath 
        • occurring along tendon sheaths of hands and feet
  • Etiology
    • now thought to be neoplastic in origin
      • previously thought to be chronic inflammation or trauma-induced hemorrhage
  • Genetics
    • 5q33 chromosomal rearrangement 
    • increased expression of CSF1 gene
  • Prognosis
    • local recurrence is common
Presentation
  • History
    • 50% of patients will have a prior history of trauma to the area
  • Symptoms
    • pain and swelling
    • mechanical pain and limited motion
    • recurrent atraumatic hemarthrosis is hallmark of disorder
  • Physical exam
    • intra-articular form (classic PVNS)
      • evaluate for joint effusion and erythema
    • extra-articular form (giant cell tumor of tendon sheath)
      • palpable masses seen along tendon sheath
      • painless
      • soft
Characteristic
Localized PVNS
Diffuse PVNS
Location
Hands > knees (anterior compartment) > ankle
Knee (75%)
Age
30-50y
<40
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
 
Imaging
  • Radiographs
    • may show cystic erosion with sclerotic margins on both sides of the joint 
  • CT
    • may show cystic erosions on both sides of the joint 
  • MRI
    • provides excellent delineation of intra-articular and extra-articular disease 
    • low signal intensity on both T1 and T2    
      • due to hemosiderin deposits
    • "blooming artifact"
      • signal loss on gradient-echo sequences
      • because of iron in hemosiderin
    • presence of fat signal (T1) within the lesion
    • can evaluate extra-articular extension of an intra-articular process
      • commonly see posterior extension outside of the knee joint
Studies
  • Arthrocentesis
    • grossly bloody effusion
  • Arthroscopy (gross appearance)
    • brownish or reddish inflamed synovium is typical of PVNS  
    • frond like pattern of papillary projections
  • Biopsy
    • synovial biopsy should be performed if there is any doubt of the diagnosis
  • Histology
    • mononuclear stromal cells infiltrating the synovium
    • highly vascular villi with hyperplastic synovial cells  
    • hemosiderin stained multinucleated giant cells  
    • pigmented foam cells (lipid-laden histiocytes)
    • mitotic figures common
Treatment
  • Nonoperative
    • observation
      • indications
        • minimal role for nonoperative treatment if disease is symptomatic
  • Operative
    •  total synovectomy (anterior arthroscopic, posterior open, for the knee)
      • classified as marginal excision
      • preferred technique
      • indications
        • in grossly symptomatic and painful disease
      • technique
        • intra-articular disease
          • techniques range from arthroscopic partial synovectomy to fully open total synovectomy
          • dependent on extent and location of disease
          • frequent recurrence is common 
          • mostly due to incomplete synovectomy
        • extra-articular
          • marginal excision is adequate for giant cell tumor of tendon sheath
          • recurrence (which is common) is treated with repeat excision
    • external beam irradiation
      • when combined with total synovectomy, reduces rate of recurrence to 10-20%
      • 30-35Gy in 15 fractions, or 50Gy in 25 fractions
Differential Diagnosis
  • Synovial chondromatosis
  • Hemophilia
  • Inflammatory synovitis
Techniques
  • Arthroscopic synovectomy for localized PVNS 
    • routine arthroscopic portals for knee, ankle, and shoulder
    • technique
      • perform as thorough resection of synovium as possible
      • excellent for focal or limited PVNS
    • postoperative
      • generally treat with some rest after procedure 
    • pros
      • minimally invasive approach
      • quick return to function
    • cons
      • unable to access the posterior portions of the joint
      • unable to address extraarticular disease
  • Arthroscopic synovectomy combined with open posterior synovectomy (knee) for diffuse PVNS
    • preferred method for diffuse
    • approach
      • posterior approach to the knee via transverse or S-shape incision across popliteal fossa
      • approach between 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
    • pros
      • allows surgeon to fully address all diseased sections
      • allows for thorough synovectomy
    • cons
      • posterior approach to the knee requires approaching neurovascular bundle
  • Total joint arthroplasty and synovectomy
    • indicated in advanced disease with severe degenerative joint changes
    • applicable to knee, hip, shoulder
  • Total synovectomy and arthrodesis
    • indicated in severe disease of the ankle
Complications
  • Recurrence
    • recurrence is the most frequent complication for both intra-articular and extra-articular disease
      • 30% recurrence rate despite complete synovectomy
        • same rates for complete open vs open+arthroscopic
      • rates can be reduced with addition of external beam radiation
  • Skin necrosis, radiation induced sarcoma
    • complications of radiation therapy
IBank
  Location
Xray
CT
CT
B. Scan
MRI
Gross
Histo(1)
Case B Knee
     

Case A

Ankle
 

 

 
 
     
 
 

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Questions (14)
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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? Review Topic

QID: 4482
FIGURES:
1

Neoadjuvant chemotherapy, surgical resection, adjuvant chemotherapy

4%

(164/4343)

2

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

4%

(158/4343)

3

Arthroscopic or open resection of the lesion

86%

(3737/4343)

4

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

3%

(116/4343)

5

Arthroscopic or open plica removal and lateral retinacular release

3%

(126/4343)

L 2

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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? Review Topic

QID: 3034
FIGURES:
1

Aneurysmal bone cyst

1%

(29/2433)

2

Periosteal osteosarcoma

1%

(36/2433)

3

Chondroblastoma

2%

(47/2433)

4

Myositis ossificans

1%

(24/2433)

5

Pigmented villonodular synovitis (PVNS)

94%

(2286/2433)

L 1

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

QID: 417
1

High grade sarcoma surrounding a major nerve

6%

(100/1698)

2

Intermediate grade solitary fibrous tumor

12%

(199/1698)

3

Atypical lipomatous tumor

9%

(158/1698)

4

Tibial adamantinoma

7%

(114/1698)

5

Pigmented villonodular synovitis

66%

(1123/1698)

L 2

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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? Review Topic

QID: 8667
FIGURES:
1

Rheumatoid arthritis

1%

(2/209)

2

Pigmented villonodular synovitis (PVNS)

84%

(175/209)

3

Synovial sarcoma

6%

(13/209)

4

Synovial chondromatosis

7%

(15/209)

5

Fibromatosis

0%

(0/209)

L 2

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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? Review Topic

QID: 1390
FIGURES:
1

Synovial sarcoma

2%

(28/1387)

2

Rheumatoid nodule

0%

(5/1387)

3

Septic arthritis

0%

(5/1387)

4

Synovial chondromatosis

4%

(50/1387)

5

Pigmented villonodular synovitis

93%

(1289/1387)

L 1

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(OBQ04.30) A 35-year-old man presents to your office complaining of vague knee pain and a fullness surrounding his patellar tendon. MRI and histology are shown. What is the name of the analogous histologic disease when located away from the synovial lining of joints? Review Topic

QID: 91
FIGURES:
1

Fibrous dysplasia

7%

(124/1781)

2

Hemangioma

12%

(206/1781)

3

Synovial sarcoma

15%

(260/1781)

4

Giant cell tumor of bone

2%

(44/1781)

5

Giant cell tumor of tendon sheath

63%

(1129/1781)

L 3

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