pigmented villonodular synovitis31may16

PIGMENTED VILLONODULAR SYNOVITIS

DR. KUMAR SUPRASHANTDNB RESIDENT

HINDU RAO HOSPITAL AND NDMC MEDICAL COLLEGE

OVERVIEW

Origin & classificationClinical featurePathologyRadiologyDifferential diagnosisDiagnosisTreatment

• 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

• 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

Classification

• LOCALIZED- identical histologically to GCT of tendon sheath

• DIFFUSE- histologically similar to localized form but involve the entire synovium

LOCALIZED PVNS• Age- 30 to 50yrs• Female preponderance• Predominantly in hand –adjacent IP joint• Foot, ankle, wrist less commonly involved

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

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

Clinical imageGross picture showing brown & yellow nodular mass

Microscopic picture with hemosiderin deposition

Radiology

• Plain radiograph–Circumscribed soft tissue mass–Cortical erosion in some cases

• MRI not useful for localized form due to variable hemorrage

Radiograph with cortical

erosion Clinical image Intraoperative image

Intraoperative image showing yellowish brown thickened nodular synovium

D/D

• Foreign body granuloma• Fibroma of tendon sheath• Necrobiotic granuloma• Tendon xanthoma• Fibrosis of tendon sheath

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

Clinical features

• Long h/o pain in joint• Swelling disproportionate to degree of pain• Joint effusion• Hemarthrosis• Locking• Limitation of movement

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

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

CT

• Lesion show high attenuation due to hemosiderin

• Useful in delineating bone cyst formation and erosion

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

Normal radiograph

MRI-Joint effusion & synovial thickening in anterior portion of joint

19 yr girl with c/o Pain & swelling of right knee

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

Anterior soft tissue mass at

ankleMRI- mass presents with intermediate

signals without bone erosion

19 yr girl with ankle pain & swelling

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

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

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

Newer advances

• Intraarticular radiation synovectomyyttrium-90, dysprosium-165

( still experimental)• TNF-α blockade therapy- in resistant cases of

PVNS

• PVNS is a benign lesion but recur locally• Local PVNS- recurrence of 10-25%• Diffuse PVNS- 15% at 5 years

35% at 25 years

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