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 Dr. Tushar Mehta

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

2 Thoughts on “PIGMENTED VILLONODULAR SYNOVITIS (PVNS)

  1. Dr GAURAV YADAV on May 15, 2013 at 11:54 AM said:

    thnx a lot sir…wonderfully explained….!!

  2. 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

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