pigmented villonodular synovitis31may16

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OVERVIEWOrigin & classificationClinical featurePathologyRadiologyDifferential diagnosisDiagnosisTreatment

Most common disorder of synoviumControversy of origin been reactive(itis) or neoplastic(oma)Reactive- evidence of trauma , multifocal originNeoplastic-autonomous growth, apoptosis resistanceMitochondrial 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, glucuronidase, naphthyl acetate esteraseImmunohistochemical- CD68, HAM56, MAC386, PG-M1

ClassificationLOCALIZED- identical histologically to GCT of tendon sheathDIFFUSE- histologically similar to localized form but involve the entire synovium

LOCALIZED PVNSAge- 30 to 50yrsFemale preponderancePredominantly in hand adjacent IP jointFoot, ankle, wrist less commonly involved

Clinical featureLong gradual progressionNon tender, firm, irregular, nodule of varying size fixed to underlying structures, free from skin (except in distal phalanx)

PVNS near PIP of index finger

pathologyGross- 0.5 -4 cm lobulated massCut section- pink gray background with yellow brown areasMicroscopy- 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

RadiologyPlain radiographCircumscribed soft tissue massCortical erosion in some casesMRI not useful for localized form due to variable hemorrage

Radiograph with cortical erosionClinical imageIntraoperative image

Intraoperative image showing yellowish brown thickened nodular synovium

D/DForeign body granulomaFibroma of tendon sheathNecrobiotic granulomaTendon xanthomaFibrosis of tendon sheath

DIFFUSE PVNSYounger ageFemale preponderanceCan involve joints & extra-articular locationsMC joint is kneeHip, ankle, foot, shoulder, wrist, sacroiliac joint, posterior elements of spine

Clinical featuresLong h/o pain in jointSwelling disproportionate to degree of painJoint effusionHemarthrosisLockingLimitation of movement

PathologyGross-absence of collagenous capsuleMicroscopy- polyhedral cells with deep blue cytoplasm(hemosiderin), polymorphic appearance due to spindle cell, xanthoma cell, giant cell. Coarse villi- shag carpet appearanceFine villi- fernlike villiAspiration-serosanguineous or blood tinged fluid

Radiology Plain radiographSoft tissue- intraarticular effusion, lobulated massBony Envade subchondral bone cyst formationMarginal & pressure erosionConcentric erosion of femoral neck- apple core deformityMarginal erosion- bubbly appearanceJoint narrowing

CTLesion show high attenuation due to hemosiderinUseful in delineating bone cyst formation and erosion

MRI- most informativeNodular 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 MRIReveals lesion not visible directly by arthoscopy such as posterior to cruciate ligament or within popliteal cyst

Normal radiographMRI-Joint effusion & synovial thickening in anterior portion of joint19 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 ankle

MRI- mass presents with intermediate signals without bone erosion

19 yr girl with ankle pain & swelling

D/DChronic hemarthrosis & hemosiderin depositionHemophilic arthopathy- classical history and invsnSynovial hemangioma- common in children and youngBone erosion, subchonral cyst, joint narrowingOsteoarthritis- characteristic osteophytesRA- polyarticular(PVNS monoarticular)Tuberculous arthritis- periarticular osteoporosis & abscess

Work up & diagnosis of PVNSClinical featureLaboratory invs- non specificRadiography- normal/ soft tissue mass/ erosionMRI- most informativeJoint aspirationBiopsyArthroscopySynovectomy

Treatment Localized form- marginal excisionDiffuse form- Total synovectomyOpen synovectomy complicated by development of painful adhesions & stiffnessArthroscopic synovectomy- t/t of choicecontraindicated for inaccessible lesionsArthroplasty considered if significant secondary degenerative changes of joint surfaces

Newer advancesIntraarticular radiation synovectomyyttrium-90, dysprosium-165( still experimental)TNF- blockade therapy- in resistant cases of PVNS

PVNS is a benign lesion but recur locallyLocal 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

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