Pigmented villonodular synovitis31may16
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Transcript of Pigmented villonodular synovitis31may16
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PIGMENTED VILLONODULAR SYNOVITIS
DR. KUMAR SUPRASHANTDNB RESIDENT
HINDU RAO HOSPITAL AND NDMC MEDICAL COLLEGE
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OVERVIEW
Origin & classificationClinical featurePathologyRadiologyDifferential diagnosisDiagnosisTreatment
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• Most common disorder of synovium• Controversy of origin been reactive(itis) or
neoplastic(oma)• Reactive- evidence of trauma , multifocal
origin• Neoplastic-autonomous growth, apoptosis
resistance• Mitochondrial dysfunction is the primary
factor in pathogenesis
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• Proliferation of both type A & type B cells (predominantly type A)
• Monocyte lineage (suggested by)
• Enzymatic- acid phosphatase, β glucuronidase, α naphthyl acetate esterase
• Immunohistochemical- CD68, HAM56, MAC386, PG-M1
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Classification
• LOCALIZED- identical histologically to GCT of tendon sheath
• DIFFUSE- histologically similar to localized form but involve the entire synovium
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LOCALIZED PVNS• Age- 30 to 50yrs• Female preponderance• Predominantly in hand –adjacent IP joint• Foot, ankle, wrist less commonly involved
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Clinical feature• Long gradual progression• Non tender, firm, irregular, nodule of varying
size fixed to underlying structures, free from skin (except in distal phalanx)
PVNS near PIP of index finger
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pathology
• Gross- 0.5 -4 cm lobulated mass• Cut section- pink gray background with
yellow brown areas• Microscopy- polyhedral cells surrounded by
collagen, giant cells ,xanthoma cells, mitotic figures, hemosiderin deposition
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Clinical imageGross picture showing brown & yellow nodular mass
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Microscopic picture with hemosiderin deposition
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Radiology
• Plain radiograph–Circumscribed soft tissue mass–Cortical erosion in some cases
• MRI not useful for localized form due to variable hemorrage
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Radiograph with cortical
erosion Clinical image Intraoperative image
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Intraoperative image showing yellowish brown thickened nodular synovium
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D/D
• Foreign body granuloma• Fibroma of tendon sheath• Necrobiotic granuloma• Tendon xanthoma• Fibrosis of tendon sheath
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DIFFUSE PVNS
• Younger age• Female preponderance• Can involve joints & extra-articular locations• MC joint is knee• Hip, ankle, foot, shoulder, wrist, sacroiliac
joint, posterior elements of spine
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Clinical features
• Long h/o pain in joint• Swelling disproportionate to degree of pain• Joint effusion• Hemarthrosis• Locking• Limitation of movement
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Pathology
• Gross-absence of collagenous capsule• Microscopy- polyhedral cells with deep blue
cytoplasm(hemosiderin), polymorphic appearance due to spindle cell, xanthoma cell, giant cell.
Coarse villi- shag carpet appearanceFine villi- fernlike villi
• Aspiration-serosanguineous or blood tinged fluid
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Radiology Plain radiographSoft tissue- intraarticular effusion, lobulated
massBony • Envade subchondral bone – cyst formation• Marginal & pressure erosion
Concentric erosion of femoral neck- apple core deformityMarginal erosion- bubbly appearance
• Joint narrowing
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CT
• Lesion show high attenuation due to hemosiderin
• Useful in delineating bone cyst formation and erosion
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MRI- most informative• Nodular thickened synovial masses throughout
joint cavity, multiple areas of hypointensity on T1 & variable intensity on T2 ( due to presence of hemosiderin)
• Blooming artifact from hemosiderin(seen on gradient echo sequence)- nearly pathognomic of PVNS at MRI
• Reveals lesion not visible directly by arthoscopy such as posterior to cruciate ligament or within popliteal cyst
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Normal radiograph
MRI-Joint effusion & synovial thickening in anterior portion of joint
19 yr girl with c/o Pain & swelling of right knee
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Extensive erosion of distal femur with sclerotic margins & normal joint space
MRI- tissue replacing
entire knee joint
MRI- cortical erosion of lower femur and upper
tibia
53 yr woman with recurrent hemarthrosis & swelling right knee
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Anterior soft tissue mass at
ankleMRI- mass presents with intermediate
signals without bone erosion
19 yr girl with ankle pain & swelling
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D/DChronic hemarthrosis & hemosiderin deposition• Hemophilic arthopathy- classical history and
invsn• Synovial hemangioma- common in children
and youngBone erosion, subchonral cyst, joint narrowing• Osteoarthritis- characteristic osteophytes• RA- polyarticular(PVNS monoarticular)• Tuberculous arthritis- periarticular
osteoporosis & abscess
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Work up & diagnosis of PVNS• Clinical feature• Laboratory invs- non specific• Radiography- normal/ soft tissue mass/
erosion• MRI- most informative• Joint aspiration• Biopsy• Arthroscopy• Synovectomy
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Treatment • Localized form- marginal excision• Diffuse form- Total synovectomyoOpen synovectomy – complicated by
development of painful adhesions & stiffness
oArthroscopic synovectomy- t/t of choicecontraindicated for inaccessible lesions
oArthroplasty considered if significant secondary degenerative changes of joint surfaces
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Newer advances
• Intraarticular radiation synovectomyyttrium-90, dysprosium-165
( still experimental)• TNF-α blockade therapy- in resistant cases of
PVNS
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• PVNS is a benign lesion but recur locally• Local PVNS- recurrence of 10-25%• Diffuse PVNS- 15% at 5 years
35% at 25 years
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ConclusionPVNS represents an uncommon benign
hypertrophic synovial process It is characterized by villous, nodular, and
villo nodular proliferation and pigmentation from hemosiderin
The MR imaging is useful for diagnosis and is optimal for identifying the extent of synovial disease, surveying and detecting recurrence.
Treatment is to remove the lesion as completely as possible
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