Pigmented villonodular synovitis

1. Pigmented villonodular
synovitis

2. • Also known as synovial xanthoma or villous synovitis is an idiopathic
villous overgrowth and pigmentation of synovial membrane.
• Sites-MC Knee
• Other sites-Hip,ankle,shoulder,elbow,spine
•
• Associated with GCT of tendon sheath(Pigmented villonodular tumor
of tendon sheath)

3. • Jaffes theory-Inflammatory response with the causative agent being
unknown.
• Overexpression of CSF 1 gene and mutation in chromosome 1p13 has
been said .
• Xanthoma cells are deriveD from reticuloendothelial system and are
modified to contain cholesterol.
• Colour is typically yellowish to yellowish brown due to cholesterol and
haemosiderin pigmentation.

4. Pathology Gross specimen showing chocolate
coloured mass having rubber like consistency

5. Microscopy

6. Clinical features
• Usually men between 20-40 years get affected with/without previous history of trauma.
• Asymptomatic in some cases
• Insidious onset of pain,swelling ,limp, stiffness.
• Occasionally snapping and locking of the joint.
•
• On examination effusion,joint line tenderness
•
• Reccurent atraumatic haemarthrosis is the hallmark of this condition.

7. Investigations
• 1)ESR,CRP-Usually normal
•
• 2)Other routine blood investigations.
• 3) Diagnostic arthroscopy followed by biopsy.It is gold standard.
• 4) Arthrocentesis/Joint aspiration-To see for orange brown colour
fluid containing cholesterol.

9. Radiological
• 1)X-ray of the affected joint-
• See for joint effusions,cystic lesions with sclerosis
• 2)MRI
• Low intensity T1 and T2
• High intensity STIR(Short inversion T1 recovery)
• Blooming artifact-Signal loss because of iron in haemosiderin.

10. Management
• Asymptomatic-Observe
• Medical-Pexidartinib(CSF 1 receptor antagonist)
• Sold under brand name turalio
• Given for these patients with extensive disease who will not respond
to surgery
• Side effect-Hepatotoxicity,deranged enzymes,loss of hair,rash.

11. • Intra Articular radiation-30-35 gy in 15 fractions Or 50 Gy in 25
fractions.
• Surgery-Arthroscopic synovectomy/Open synovectomy of the knee.

12. Complications
• 1) Reccurence
• 2)Joint destruction
• 3)skin necrosis
• 4)Radiation induced sarcoma

13. • Thank you