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Pigmented Villonodular Synovitis

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Pigmented Villonodular Synovitis, PVNS, Diffuse Giant Cell Neoplasm of Tenosynovium, Chronic Hemorrhagic Villous Synovitis

  • See Also
  1. Monoarthritis
  2. Joint Pain
  • Epidemiology
  1. Onset in young adults (uncommon in children)
  2. Genders are equally affected
    1. Women may have a slightly higher Incidence
  3. Incidence: 9.2 per million in U.S. per year
  • Pathophysiology
  1. Aggressive, Tenosynovial Giant Cell Tumor affecting synovial lining of joints and tendons
  2. Most often associated with a Chromosome 1p13 translocation
  3. Overexpression of Colony Stimulating Factor 1 (CSF1)
    1. Results in focal regions of hyperplasia within synovium lining joints and tendons
  • Precautions
  1. Commonly misdiagnosed as Osteoarthritis, resulting in delayed diagnosis (18 months on average)
  • Symptoms
  1. Gradual onset of Joint Pain and swelling
  2. Progresses to severe pain and reduced joint range of motion
  • Signs
  1. Characteristics
    1. Joint effusion
    2. Painful and reduced joint range of motion
  2. Distribution
    1. Most common as a gradual onset Monoarthritis (however Polyarthritis may occur)
    2. Most commonly affects the knee, hip and Ankle Joints
      1. Knee infrapatellar fat pad is most commonly affected
  • Imaging
  1. XRay
    1. Bony erosions within the affected joint
  2. MRI
    1. Joint effusion
    2. Hemosiderin deposits
    3. Synovial expansion
    4. Bony erosions
  • Labs
  1. Inflammatory markers (CRP, ESR)
    1. Typically normal
  2. Arthrocentesis
    1. Dark brown or hemorrhagic coloration to Synovial Fluid
  3. Histology
    1. Synovial-like mononuclear cells
    2. Hemosiderin-laden Macrophages
    3. Foam cells
    4. Inflammatory cells
    5. Multinucleated Osteoclast-like giant cells
  • Differential Diagnosis
  1. See Joint Pain
  2. See Monoarthritis
  • Management
  1. Total synovectomy of the affected joint (arthroscopy preferred)
    1. Historically, most common and effective treatment, but high recurrence rate
  2. Radiation Therapy (external beam, 30-50 Gy)
    1. Highly effective, even as monotherapy, and especially in combination with synovectomy
  3. Biologic Agents
    1. Emactuzumab binds CSF1 receptor and has been investigated for use in PVNS
  • Prognosis
  1. Best outcomes with early treatment (greater joint destruction with delayed diagnosis)
  2. High recurrence rate after treatment approaches 50% (better when Radiation Therapy is added to regimen)
  3. Left untreated, PVNS results in severe joint dysfunction and deformity
  • References
  1. Fecek (2023) Pigmented Villonodular Synovitis, StatPearls
    1. https://www.ncbi.nlm.nih.gov/books/NBK549850/
  2. Staals (2016) Eur J Cancer 63:34-40 +PMID: 27267143 [PubMed]