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%.

3 comments

  1. 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

  2. 3.0 T MRI OF RIGHT WRIST:
    MR imaging of the right wrist was performed using spin-echo and gradient-echo [GRASS] pulse sequences and high resolution 3.0mm T1-and T2 weighted serials sections were obtained in the sagittal and coronal planes using a dedicated phased array coil on a 3.0 Tesla scanner with high strength gradient.

    Tablet marker was placed at site of swelling.

    Alignment: Normal

    Fluid:
    Carpus effusion: Mild joint effusion with a loculated component is seen at radio-dorsal aspect.
    Distal radioulnar joint effusion: mild joint effusion

    Intrinsic ligaments:
    Scapholunate: Intact
    Lunotriquetral: Intact

    Ulnar side:
    Triangular fibrocartilage: Partial central perforation is seen involving articular disc of triangular fibrocartilage is noted.
    Intact peripheral attachment of triangular fibrocartilage is noted.

    Extensor compartment:
    I: Normal.
    II: Normal.
    III: Normal.
    IV: Normal.
    V: Normal.
    VI: Normal.
    No evident tendinosis or tenosynovitis or tear.

    Flexor compartment:

    Carpal tunnel:
    Median nerve: Normal appearance of median nerve. No evident abnormal flattening or abnormal signal intensity

    Flexor retinaculum: Normal. No evident abnormal palmar bowing is seen
    Flexor tendons: Normal. No evident tenosynovitis.

    Guyon canal: Normal appearance of ulnar nerve and vessels. No evident ganglion cyst.

    Bones & Joints : A welldefined lobulated altered signal intensity lesion is noted at volar aspect of radio-carpal joint and distal radius, deep to flexor carpi radialis tendon. It appears markedly hypointense on T1W & T2WI suggests ? haemosiderin ?? calcification. However, screening CT does not show calcification / ossification at above site (density: 70 HU).
    It is causing mild cortical irregularity at volar aspect of distal radius and edematous changes in pronator quadraus muscle.
    Radial artery is noted at lateral aspect of the lesion.
    It measures 18 x 10 mm in axial plane with craniocaudal extent of 22 mm.

    Another similar characteristic loose body is also noted at volar and medial aspect of hamate bone, deep to piso-humate and piso-metacarpal ligament (insertion of flexor carpi ulnaris tendon).
    It measures 16 x 6 mm in axial plane and 10 mm in sagittal plane.
    No evident osseous erosion is seen.

    Piso-triquetral joint effusion is noted with another small loose body within at inferior aspect. It measures 6.9 x 2.4 mm in axial plane with craniocaudal extent of 4 mm.

    Mild wrist joint effusion with a loculated component is seen at radio-dorsal aspect.

    In view of multiple similar characteristic lobulated lesions in relation to wrist joint as described above, possibility of synovial pathology appears most likely.
    Differential considerations would be of :
    Pigmented villonodular synovitis.
    Primary synovial chondromatosis.

    Thumb carpometacarpal joint: Normal. No evident changes of osteoarthritis.
    Scaphotrapezotrapezoidal joint: Normal. No evident changes of osteoarthritis.
    Hamate-lunate: Normal. No evident chondromalacia or changes of osteoarthritis.

    Muscles: Normal.

    Vessels: Normal.

    IMPRESSION:
    The MR findings are:
    Mild wrist joint effusion with a loculated component at radio-dorsal aspect.
    Discrete T1/T2 hypointense lesions ( n=3) at volar aspect of radiocarpal joint & distal radius – deep to flexor carpi radialis ; at volar & medial aspect of hamate bone – deep to flexor carpi ulnaris tendon) and at inferior aspect of piso-triquetral joint as described above.
    In view of multiple similar characteristic lobulated lesions in relation to wrist joint as described above, possibility of synovial pathology appears most likely.
    Differential considerations would be of :
    Pigmented villo-nodular synovitis.
    Primary synovial chondromatosis.
    Partial central perforation involving articular disc of triangular fibrocartilage. Intact peripheral attachment of triangular fibrocartilage.
    No evidence of tendinosis or tear or tenosynovitis.

    I OPERATED THE WRIST SIDE BUT STILL PAIN IN OTHER SIDE IS THERE ANY SALUTATION PLEASE UPDATE US

    THANKS RAJKUMAR

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