Pigmented villonodular synovitis

Pigmented villonodular synovitis by Dr. Bipul Borthakur

1. PIGMENTED VILLONODULAR SYNOVITIS By: Dr. Bipul Borthakur Professor, Dept of Orthopaedics, SMCH

2. DEFINITION Pigmented Villonodular Synovitis (PVNS) is a slow growing tumor lesion of uncertain etiology arising from the synovial membrane , characterized by pigmented villous and nodular outgrowths of the synovial membrane of the bursae or tendon sheath Since bursae and tendon sheaths are related to synovium in origin, they too are sites of xanthomatous growth

3. PREVALENCE Age: 3 rd to 4 th decade of life, rare in children Sex: no sex based predilection Incidence: 1.8 per million Appendicular skeleton, especially large joints such as knee and hip joints are frequently involved No predilection for any laterality

4. ETIOPATHOGENESIS

5. ETIOPATHOGENESIS It is a reactive condition , and not a true neoplasm Recurrent atraumatic haemarthrosis is a characteristic feature Often aggressive , with marked extra- articular extension

6. TYPES: INTRA AND EXTRA-ARTICULAR INTRAARTICULAR Monoarticular involvement ( most common), mimicking arthritis Granowitz described two forms: Localised form Diffuse form

7. Localized form Focal involvement of the synovium Nodular/ sessile or pedunculated well circumscribed masses Diffuse form More common Affect virtually the entire synovium EXTRAARTICULAR Tendon sheath and bursae PVNS

8. SITES MC site: knee > hip > shoulder Knee: Anterior compartment commonly involved Mostly menisco -capsular junction MC site: synovium in the region of anterior horn of medical meniscus Infrapatellar fat pad, suprapatellar pouch, intercondylar notch, anterior horn of lateral meniscus, and medial and lateral recesses of knee Uncommon: elbow, ankle, foot, wrist Rare: spine, tendon sheaths

9. CLINICAL FEATURES Symptoms Signs Pain : 80% Swelling : 75% Stiffness Locking catching Instability Palpable mass : 12% Effusion – floating patella Tenderness Decreased ROM Usually not palpable; In localised variety, can show a joint mouse

10. TYPE SPECIFIC FEATURES LOCALIZED PVNS Initially painless If untreated, caused continuous pain and discomfort, limiting activities of daily living At knee, mostly presents with signs and symptoms of meniscal pathology Locking Catching Instability Well circumscribed soft tissue mass

11. DIFFUSE PVNS Slow, insidious onset pain Swelling Stiffness Most of or all of the joint involved Decreased ROM Poorly localised / ill defined mass May encroach on nearby neurovascular structures Osteoarthritis : continued inflammation and joint erosions lead to articular cartilage destruction

12. INVESTIGATION : X-RAY Soft tissue swelling Cysts, bony erosions in joint- mimicks gout . Osteoporosis is characteristically absent Can affect epiphysis in children

13. Reciprocal bony lesions on opposite sides of joint, despite articular preservation- highly suggestive Degenerative changes, osteoarthritic changes

14. MRI Investigation of choice LPVNS: Nodular mass ( periarticular or synovial) bone erosion DPVNS: extensive mass and thickening of joint lining destruction of bone and cartilage

15. ULTRASONOGRAPHY Loculated joint effusion Complex heterogenous echogenic masses with markedly thickened synovium ASPIRATION Blood tinged thick orange brown fluid containing cholesterol in large amounts

16. ARTHROSCOPY Diagnostic and therapeutic value Direct visualisation of synovium Normal arthroscopic findings however does not exclude PVNS

17. HISTOPATHOLOGY Synovium looks like a “ shaggy carpet ” Histiocytes lipid laden macrophages hemosiderin containing cells frequent giant cells Subsynovial nodular proliferation of large round, polyhedral or spindle cells with prominent cytoplasm and pale nuclei

18. DIFFERENTIAL DIAGNOSIS Hemophilic lobular synovitis Menisceal tear or other ligamentous injury Synovial chondromatosis Osteoarthritis Gouty arthritis Rheumatoid arthritis

19. TREATMENT Local excision- for nodular form Total Synovectomy - arthroscopic or open Radiotherapy Advanced cases : Arthrodesis / arthroplasty + extensive synovectomy

20. SYNOVECTOMY Open : Anterior approach or medial parapatellar approach : for the diffuse form Posterior approach done subsequently for extensions into the popliteal fossa

21. SYNOVECTOMY Arthroscopic: Gained popularity because of several advantages over open technique Preferred for LPVNS Higher recurrence rate in DPVNS Vascular or neurological injury : especially in posterior extra- articular extension or fibrosis after irradiation

22. RADIOTHERAPY 3500- 4000 cGy Radiation induced synovectomy Intra- articular radiation synovectomy with Yttrium-90 Use : Recurrence

23. PROGNOSIS LPVNS: DPVNS: Excellent prognosis Low recurrence rate: 8% Complete surgical excision is difficult Recurrence rate: upto 46%

24. THANK YOU “ yajñārthātkarmaṇo’nyatra loko’yaṃ karmabandhanaḥ tadarthaṃ karma kaunteya muktasaṅgaḥ samācara ” “Man is bound by his own action except when it is performed for the sake of sacrifice. Therefore, Arjuna , efficiently perform your duty, free from attachment, for the sake of sacrifice alone .”