Pigmented villonodular synovitis

Pigmented villonodular synovitis

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  Pigmented villonodular synovitis
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  • Also knownas 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)
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  • Jaffes theory-Inflammatoryresponse 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.
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  Pathology Gross specimenshowing chocolate coloured mass having rubber like consistency
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  Microscopy
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  Clinical features • Usuallymen 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.
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  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.
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  Radiological • 1)X-ray ofthe 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.
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  Management • Asymptomatic-Observe • Medical-Pexidartinib(CSF1 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.
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  • Intra Articularradiation-30-35 gy in 15 fractions Or 50 Gy in 25 fractions. • Surgery-Arthroscopic synovectomy/Open synovectomy of the knee.
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  Complications • 1) Reccurence •2)Joint destruction • 3)skin necrosis • 4)Radiation induced sarcoma
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