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Synovitis, Pigmented Villonodular

Synovitis, Pigmented Villonodular is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

  • Pigmented villonodular synovitis (PVNS), also known as tenosynovial giant cell tumor, is a benign proliferative and inflammatory disease associated with synovial hypervascularity.
  • Classically, PVNS presents with a single nodule or multiple nodules along a synovial layer or tendon sheath containing hemosiderin deposits, macrophages, and multinucleated giant cells.
  • Two forms of PVNS exist.
    • Diffuse pigmented villonodular synovitis (DPVNS)
    • Localized pigmented villonodular synovitis (LPVNS)-focal pedunculated lesions

Epidemiology

Incidence
  • 1.8/1 million cases/year
  • Predominant age: 20 to 50 years
  • There are no definite environmental, genetic, ethnic, or occupational associations; however, a history of preceding trauma with bleeding into the affected joint has been observed.

Geriatric Considerations
Rare in elderly patients

Pediatric Considerations
Rare in children

Etiology and Pathophysiology

  • Exact cause is unknown.
  • History of trauma is noted in up to 56% of patients with PVNS.
  • Hypothesized to be caused by joint trauma with bleeding into joint with subsequent development of hemosiderin deposits and inflammatory markers causing joint destruction
  • Recurrent bleeding into joint spaces leading to cartilage destruction and osteoarthritis is the natural progression in diseases such as hemophilia, which supports the above hypothesis; however, the pathophysiology is still unclear.
    • Histologically, LPVNS and DPVNS have similar features: hemosiderin-loaded macrophages give the typical brown or “pigmented” color.
    • Multinucleated giant cells with osteoclastic features
    • Lipid-laden macrophages
  • DPVNS and LPVNS differ in their disease course.
    • DPVNS (more common than LPVNS)
      • Characterized by involvement of most or all the joint synovium with multiple nodules along synovial layer or tendon sheath with associated edema and pain
      • Rapidly destructive, with poorer prognosis compared to LPVNS
      • Potentially encroaches on neurovascular structures due to extraarticular progression complicating surgical resection, however, usually remains intra-articular
      • Continued inflammation and joint erosions lead to articular cartilage destruction and subsequent osteoarthritis.
    • LPVNS
      • Characterized by a single discrete or pedunculated nodule
      • Favorable prognosis due to localized nature
      • Low recurrence rate postoperatively

Genetics
  • t(1;2)(p12;q35) is a translocation of collagen 6A3 (COL6A3) gene and macrophage colony-stimulating factor 1 (CSF1) gene. It has been found in a fraction of tumor cells.
  • This mutation causes an overexpression of CSF1, which activates and recruits its receptor (CSF1R), which is usually found in monocytes and macrophages in PVNS nodules.

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Citation

Stephens, Mark B., et al., editors. "Synovitis, Pigmented Villonodular." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816221/all/Synovitis__Pigmented_Villonodular.
Synovitis, Pigmented Villonodular. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816221/all/Synovitis__Pigmented_Villonodular. Accessed April 19, 2019.
Synovitis, Pigmented Villonodular. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816221/all/Synovitis__Pigmented_Villonodular
Synovitis, Pigmented Villonodular [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 April 19]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816221/all/Synovitis__Pigmented_Villonodular.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Synovitis, Pigmented Villonodular ID - 816221 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816221/all/Synovitis__Pigmented_Villonodular PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -