«

»

May 15

PIGMENTED VILLONODULAR SYNOVITIS (PVNS)

PIGMENTED VILLONODULAR SYNOVITIS (PVNS)

PIGMENTED VILLONODULAR SYNOVITIS (PVNS)

 

 

 

PIGMENTED VILLONODULAR SYNOVITIS (PVNS)

Definition:
- Pigmented villonodular synovitis (PVNS) is a slow growing lesion of uncertain etiology arising from the synovial membrane, characterized by villous and nodular overgrowths of the synovial membrane of the bursa or the tendon sheath.
- The appendicular skeleton, especially large joints such as the knee and hip joints are frequently involved.
- Synonyms: Until Jaffe in 1941 proposed the term pigmented villonodular synovitis this condition has been known as synovial xanthoma, synovial endothelioma/ fibroendothelioma, Benign fibrous histiocytoma, xanthomatous GCT, Myeloplaxoma, fibrohemosideric sarcoma.

 

Prevalance:

• Sex: no sex based predilection
• Age: 3rd-4th decades of life, rare in children
• Incidence: 1.8 per million population

 

Etiopathogenesis:
• Repetitive trauma (50%) causing recurrent local hemorrhage to affected joint (cf:hemophilics show progressive erosive arthropathies).
• Proliferation of the synovium of joints, tendon sheaths or bursae.
• It is a reactive condition, and not a true neoplasm.
• PVNS classically presents as a monoarticular disease, mimicking arthritis.
• Recurrent atraumatic haemarthrosis is a characteristic feature.

Types:

Monoarticular involvement (most common), occurs in two forms: localized and diffuse.
Two variants as described by Granowitz -

  1. Localized form (LPVNS): focal involvement of the synovium
    - Nodular / Sessile or Pedunculated masses.
    - Hands & feet
  2. b. Diffuse form (DPVNS) (more common): affects virtually the entire synovium, eg.
    - Intra-articular PVNS tends to be of the diffuse form.
    - Tendon sheath PVNS (Giant cell tumour of tendon sheath[GCCTS]), the nodular form

 

Sites:
• MC site: knee joint, followed by the hip and shoulder.
• Knee:
- anterior compartment common
- mostly at meniscocapsular junction
- synovium in the region of the anterior horn of the medial meniscus is the most common site
- infrapatellar fat pad, suprapatellar pouch, intercondylar notch, anterior horn of the lateral meniscus, and the medial and lateral recesses of the knee have been reported.
• Uncommon : elbow, ankle, shoulder, foot, wrist

 

Clinical features:
o Pain
o Swelling
o Reduced range of movement
o Locking
o Instability/palpable mass

 

 

 

Investigation:
Aspiration of joint: characteristically reveals a blood tinged brownish-stained aspirate.

X-ray:
• Soft tissue swelling will be marked due to haemorrhage and lobulated synovial tissue.
• May reveal cysts or erosions in the joint mimicking gout.
• Bony erosions are usually from without, especially in the hip
• Periarticular erosions, with a thin rim of reactive bone
• Late feature of joint space narrowing indicates articular cartilage loss, is difficult to distinguish from primary OA

MRI:
• Ideal investigation
• Nodular mass (periarticular or synovial) with bone erosion

Sonography:
• Loculated joint effusions, Complex heterogeneous echogenic masses and markedly thickened synovium

Arthroscopy:
• Direct visualisation of synovium
• Has both diagnostic and therapeutic value in resection of tumours

 

Histolopathology:
• LPVNS is pedunculated, lobular lesion localized to one area of the synovium.
• On microscopy, Histiocytes, lipid laden macrophages, hemosiderin containing cells and frequent giant cells are seen

 

Treatment:
Synovectomy:
o Total synovectomy (open or arthroscopic):
- Open (anterior approach midline incision or medial parapatellar arthrotomy) for the diffuse form for the intraarticular component
- Arthroscopic synovectomy, has gained popularity, has several advantages over the open technique, preferred for LPVNS, shows higher recurrence in DPVNS.

Radiotherapy (3500- 4000 cGy) (Radiation induced synovectomy/ intra-articular radiation synovectomy using yttrium Y-90) has been used in the management of recurrences with varying success

 

Prognosis:
• LPVNS: excellent prognosis, low recurrence rate if managed surgically, recurrence 8%.
• DPVNS: surgical excision difficult, recurrence rate of up to 46%.

About the author

Dr. Tushar Mehta

MBBS , DNB (Ortho), MNAMS, PGDCR,
http://www.facebook.com/orthopaedicsplus.in

2 comments

  1. Dr GAURAV YADAV

    thnx a lot sir…wonderfully explained….!!

  2. Lia

    Dear Dr. Mehta,
    Thank you for this website.
    I have PVNS in my elbow. How do you know that it is reactive and comes from repetitive trauma? I don’t recall hurting my elbow repeatedly. In my case, is it just an overgrowth of synovium? How does that happen?
    Thank you,
    Lia

Leave a Reply

Your email address will not be published. Required fields are marked *