Pigmented Villonodular Synovitis

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

advertisement

II. 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

III. 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

IV. Precautions

  1. Commonly misdiagnosed as Osteoarthritis, resulting in delayed diagnosis (18 months on average)

V. Symptoms

  1. Gradual onset of Joint Pain and swelling
  2. Progresses to severe pain and reduced joint range of motion

VI. 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

VII. Imaging

  1. XRay
    1. Bony erosions within the affected joint
  2. MRI
    1. Joint effusion
    2. Hemosiderin deposits
    3. Synovial expansion
    4. Bony erosions

VIII. 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

IX. Differential Diagnosis

  1. See Joint Pain
  2. See Monoarthritis

X. 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

XI. 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

XII. 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]

Images: Related links to external sites (from Bing)

These images are a random sampling from a Bing search on the term "Pigmented Villonodular Synovitis." Click on the image (or right click) to open the source website in a new browser window. Search Bing for all related images

Related Topics in Hematology and Oncology

advertisement