Pigmented Villonodular Synovitis (PVNS)

 

Epidemiology:

    • 20s to 40s

 

Common sites:

    • knee

 

Gross features:

    • involves one or more joints diffusely
    • red-brown to mottled orange-yellow
    • joint synovium is tangled, folded, with finger-like projections and nodules
    • aggressive tumours may invade adjacent bone and soft tissues

 

Histologic features:

    • often papillary architecture
    • polyhedral, moderate-sized tumour cells resembling synoviocytes
      • mild atypia
    • spreading along synovial surface and infiltrating the subsynovial compartment
    • hemosiderin deposits
    • foamy macrophages
    • multinucleated giant cells
    • zones of sclerosis

 

Immunophenotype:

Marker:

Sensitivity:

Specificity:

 

 

 

 

Molecular features:

    •  

 

Other features:

    • true neoplasm of synovial lining of joints
    • similar to to Giant Cell Tumour of Tendon Sheath (GCT)
    • significant recurrence rate
    • often history of trauma

 

References:

    • Robbins 2005