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Pigmented villonodular synovitis of the temporomandibular joint – computed tomography and magnetic resonance findings: a case report

Il-Kyu Kim, Hyun-Young Cho, Hyun-Woo Cho, Ji-Hoon Seo, Dong-Hwan Lee, Wang Peng

Department of Oral and Maxillofacial Surgery, Inha University College of Medicine, Incheon, Korea

Abstract (J Korean Assoc Oral Maxillofac Surg 2014;40:140-146)

Pigmented villonodular synovitis (PVNS) is a benign but locally aggressive and destructive disease originating in the synovial membranes. It is a pro-liferative disorder of unknown etiology. Involvement of the temporomandibular joint (TMJ) is very rare. Computed tomography clearly reveals areas of lytic bone erosion and sclerosis, and also clearly defines the extent of the tumor which is the focal areas of hyperdensity within the soft-tissue mass. Magnetic resonance images invariably show profound hypointensity on both T1- and T2-weighted sequences due to hemosiderin pigmentation. Ad-ditionally, high signal intensity on T2-weighted images may indicate cystic loculation of the joint fluid. This case study describes a rare case of PVNS of the TMJ with bone destruction of the mandibular condyle. Complete surgical excision of the lesion was performed through a preauricular approach with temporal extension. During the 10-year follow-up, two more operations were performed due to local recurrence and the fracture of the reconstruc-tion plate. Total joint reconstruction with Biomet was finally performed, and the absence of disease was confirmed with a biopsy report showing fibro-sis with hyalinization and mild inflammation of the excised soft tissue from the old lesion.

Key words: Temporomandibular joint, Pigmented villonodular synovitis, Mandibular reconstruction[paper submitted 2014. 4. 13 / revised 2014. 5. 27 / accepted 2014. 5. 28]

lessfrequentlythediseaseinvolvesthehip,shoulder,ankle,

orwrist1,2.Althoughanyjointcanbeaffected,involvementof

thetemporomandibularjoint(TMJ)isreportedlyveryrare3,4.

Lapayowkeretal.5firstdescribedPVNSoftheTMJin1973.

Accordingtooneliteraturesearch,since1973,atotalof45

PVNSpatientswithTMJinvolvementhavebeenreportedto

date6.

Inthisreport,wedescribeararecasewith10yearsoffol-

low-upofPVNSoftheTMJthatpresentedasanaggressive

tumorwithlocalrecurrence.Wealsodiscussandreviewthe

previousliteratureonPVNSoftheTMJ.

II. Case Report

InMay2004,a38-year-oldKoreanmanwasreferredto

theDepartmentofOralandMaxillofacialSurgeryatInha

UniversityHospital(Incheon,Korea)witha2-yearhistoryof

painlessswellingintherightpreauriculararea.Reviewofhis

medicalhistoryrevealedschizophreniaanddiabetesmellitus,

whichwerewellcontrolledwithmedication.Physicalexami-

nationrevealedanill-defined,hardmassapproximately3×

4cmindiameterthatwasmildlytenderonpalpationinthe

I. Introduction

Pigmentedvillonodularsynovitis (PVNS) isabenign,

but locallyaggressive,proliferativedisorderaffectingthe

synovium,oftenwithinfiltrationand/orosteoinvasion1-4.It

commonlyarisesinthetendonsheathsandlongbonejoints

andrarelyarisesinthebursae.Jaffeetal.1 introducedthe

termPVNSin1941todescribeayellowtumor-likesynovial

lesion.TheydescribedPVNSasbothlocalized(affectsonly

aportionofthejointliningortendonsheathwithasolitary

pedunculatednodular lesionwithnovilli)andmorecom-

monlydiffuse(affectstheentiresynovialjointmembraneor

bursa).Themostcommonlyaffectedjointistheknee,and

CASE REPORT

Il-Kyu KimDepartment of Oral and Maxillofacial Surgery, Inha University College of Medicine, 27 Inhang-ro, Jung-gu, Incheon 400-711, KoreaTEL: +82-32-890-2470 FAX: +82-32-890-2475E-mail: kik@inha.ac.kr

This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Copyright Ⓒ 2014 The Korean Association of Oral and Maxillofacial Surgeons. All rights reserved.

http://dx.doi.org/10.5125/jkaoms.2014.40.3.140pISSN 2234-7550·eISSN 2234-5930

ThisworkwassupportedbytheInhaUniversityResearchGrant.

Pigmented villonodular synovitis of the temporomandibular joint

141

rightpreauriculararea.Maximummouthopeningwaslim-

itedto30mm,andmandibularopeningwasaccompaniedby

slightdeviationtotheright.Therewasnoevidenceoffacial

nerve-relatedweakness,andahistoryoffacialtraumawas

notnoted.

Fig. 1. The panoramic view shows the lobulated osteolytic lesion in the right condylar head.Il-Kyu Kim et al: Pigmented villonodular synovitis of the temporomandibular joint – computed tomography and magnetic resonance findings: a case report. J Korean Assoc Oral Maxillofac Surg 2014

Fig. 3. The axial (A) and coronal (B) T2-weighted images of magnetic reso-nance imaging show the multilobulated mass-like lesion with inner high signal intensity and outer low signal intensity. Il-Kyu Kim et al: Pigmented villonodular synovitis of the temporomandibular joint – computed tomogra-phy and magnetic resonance findings: a case report. J Korean Assoc Oral Maxillofac Surg 2014

Fig. 2. The axial (A) and coronal (B) views of computed tomography show a lobulated osteolytic lesion with the bony erosion (arrow) in the mandibular condyle.Il-Kyu Kim et al: Pigmented villonodular synovitis of the temporomandibular joint – computed tomogra-phy and magnetic resonance findings: a case report. J Korean Assoc Oral Maxillofac Surg 2014

Fig. 4. The axial T1-weighted image of magnetic resonance im-aging shows the intermediate signal intensity with peripheral low signal intensity rim. Il-Kyu Kim et al: Pigmented villonodular synovitis of the temporomandibular joint – computed tomography and magnetic resonance findings: a case report. J Korean Assoc Oral Maxillofac Surg 2014

J Korean Assoc Oral Maxillofac Surg 2014;40:140-146

142

suspicionofPVNS,orfibrousorinflammatorypseudo-tumor

oftheTMJ,surgicalexplorationoftherightTMJ,excisionof

themass,andreconstructionofthemandibularcondylewere

alsoplanned.

Thesurgerywasperformedundergeneralendotracheal

anesthesia.Thezygomaticarchandthejointcapsulewere

exposedbyapreauricularincisionwithatemporalextension.

Acompletecapsulectomywasperformedforexcisionof

themassinvolvingthecondylarheadandarticulardisk.The

surfacesof theglenoidfossaandarticulareminencewere

foundtobenormal.Afterreconstructionofthecondylewith

atitaniumcondylarheadandplate,thesurgicalwoundwas

sutured.

Theexcised lesionwas4.0×3.0×2.5cminsizeand in-

Apanoramicradiographshowedalobulatedcysticoros-

teolyticradiolucencyoftherightmandibularcondyle.(Fig.1)

Acomputedtomogram(CT)showedalobulatedosteolytic

andbonyerosionintherightmandibularcondyle.(Fig.2)

Magneticresonanceimaging(MRI)showedalobulated-ap-

pearingenhancingsofttissuemassoftherightTMJandman-

dibularcondyle.The3.0×2.5cmirregularlyshapedmasshad

aheterogeneousT2signalintensityandisosignalintensityon

T1-weightedMRI.(Figs.3-5)Peripherallowsignalintensity

areaswerenotedonbothT1-andT2-weightedmagnetic

resonance(MR) images.Fineneedleaspirationcytology

wasdone.Thesmearrevealedafewclusteredandscattered

plumpspindlecellscontaininghemosiderin-pigmentsand

severalscatteredmultinucleatedgiantcells.(Fig.6)Witha

Fig. 5. The axial (A) and coronal (B) fat suppression T1-weighted images of magnetic resonance imaging show the multilobulated high signal intensity with the high soft tissue enhancement. Il-Kyu Kim et al: Pigmented villonodular synovitis of the temporomandibular joint – computed tomogra-phy and magnetic resonance findings: a case report. J Korean Assoc Oral Maxillofac Surg 2014

Fig. 6. Smear shows a few clustered and scattered plump spindle cells containing hemosiderin pigments (Papanicolaou staining, ×400).Il-Kyu Kim et al: Pigmented villonodular synovitis of the temporomandibular joint – computed tomography and magnetic resonance findings: a case report. J Korean Assoc Oral Maxillofac Surg 2014

Fig. 7. Grossly, the specimen is consisted of a few fragments of brownish gray soft tissue, cartilage and bone. Il-Kyu Kim et al: Pigmented villonodular synovitis of the temporomandibular joint – computed tomography and magnetic resonance findings: a case report. J Korean Assoc Oral Maxillofac Surg 2014

Pigmented villonodular synovitis of the temporomandibular joint

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Fig. 8. The mass is composed of many mononuclear histiocytic cells and irregularly interspersed multinucleated giant cells. Hemosiderin deposits are found (H&E staining, A: ×100, B: ×400).Il-Kyu Kim et al: Pigmented villonodular synovitis of the temporomandibular joint – computed tomography and magnetic resonance findings: a case report. J Korean Assoc Oral Maxil-lofac Surg 2014

Fig. 9. The axial (A) and coronal (B) views of computed tomography show the round soft tissue opacity on the condylar fossa of temporal bone (ar-row) and the skull base erosion with bony perforation (arrow head). Asterisk: metal condylar head.Il-Kyu Kim et al: Pigmented villonodular synovitis of the temporomandibular joint – computed tomogra-phy and magnetic resonance findings: a case report. J Korean Assoc Oral Maxillofac Surg 2014

Fig. 10. The axial (A) and coronal (B) fat suppression T1-weighted images of magnetic resonance imaging show the lobulated high signal intensity mixed up inner intermediate signal intensity with the moderate enhancement. The skull base perforation (arrow) is also seen.Il-Kyu Kim et al: Pigmented villonodular synovitis of the temporomandibular joint – computed tomogra-phy and magnetic resonance findings: a case report. J Korean Assoc Oral Maxillofac Surg 2014

J Korean Assoc Oral Maxillofac Surg 2014;40:140-146

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sionoftheskullbaseofthetemporalbonewasnoticedto

accompanytheperforation.(Figs.9,10)ThefindingsonMRI

weresimilarwiththepreoperativesignalintensitiesofthe

T1W1,T2W1,andfatsuppressionT1W1images.(Figs.10-

12)SurgerywasperformedagainonJuly2008andtherecur-

renceofPVNSwasconfirmed.Duringthefollow-upwith

CTandMRIexamination,fractureofthereconstructionplate

wasnoticed,withthepatientcomplainingofsomediscomfort

andalimitationinmouthopening.InDecember2012,total

jointreconstructionwithBiometstockprosthesis(Biomet,

Jacksonvill,FL,USA)wasperformed,andbiopsyof the

excisedsofttissuefromtheoldlesionrevealedfibrosiswith

hyalinizationandmildinflammation.Therewerenoclinical

orimagingsignsofrecurrenceafter18monthsoffollow-up

followingthefinaloperation.(Fig.13)

III. Discussion

PVNSisan idiopathicproliferativedisorder involving

thesynoviumofthejoint,tendonsheaths,andbursae.More

volvedthemandibularcondyle,whichwaserodedandperfo-

ratedonitsposteriorsurface.(Fig.7)Grossly,thespecimen

consistedofafewfragmentsofbrownishgraysoft tissue,

cartilage,andbone.Microscopically, thetumorwascom-

posedofmanymononuclearhistiocyticcellsandirregularly

interspersedmultinucleatedgiantcells.Hemosiderinpig-

mentswerealsofound.Somefoamyhistiocyticcellswere

individuallyinterspersedorformedclusters.(Fig.8)Osseous,

cartilaginous,andsofttissueinvolvementwasalsoseen.The

lesionwasdiagnosedasaPVNSwithinvolvementof the

bone,cartilage,andadjacentsofttissue.

The4-yearfollow-upCTandMRIexaminationrevealed

arecurrenceofthePVNSwitha2.3×2.1×2.6cmmass.Ero-

Fig. 13. The panoramic view after 18 months of final operation later. Il-Kyu Kim et al: Pigmented villonodular synovitis of the temporomandibular joint – computed tomography and magnetic resonance findings: a case report. J Korean Assoc Oral Maxillofac Surg 2014

Fig. 11. The axial T1-weighted image of magnetic resonance imaging shows the isosignal intensity mixed up inner low signal intensity with peripheral low signal intensity rim.Il-Kyu Kim et al: Pigmented villonodular synovitis of the temporomandibular joint – computed tomography and magnetic resonance findings: a case report. J Korean Assoc Oral Maxillofac Surg 2014

Fig. 12. The axial (A) and coronal (B) T2-weighted images of magnetic reso-nance imaging show the heterogenous high signal intensity mixed up inner low signal intensity with peripheral low sig-nal intensity rim.Il-Kyu Kim et al: Pigmented villonodular synovitis of the temporomandibular joint – computed tomogra-phy and magnetic resonance findings: a case report. J Korean Assoc Oral Maxillofac Surg 2014

Pigmented villonodular synovitis of the temporomandibular joint

145

thetumorwellbecausethetumorhasallormostofitsfocal

areasofhyperdensitywithinthesoft-tissuemassthatfurther

enhancesafteradministrationofcontrastmaterial.CTen-

hancesthethickenedsynoviumandareasofhighattenuation

causedbyirondeposits11-13.Inthispatient,CTalsoshoweda

lobulatedosteolyticlesionaccompanyingthebonyerosion,

butdidnotfindbonysclerosisandareasoffocalhyperden-

sitywithinthesofttissue.(Figs.2,9)

TheappearanceofPVNSonMRimagecanbevariable,

dependingontherelativeproportionoflipids,hemosiderin,

fibrousstroma,pannus,fluids,andcellularelements2,13.For

example,dependingonthedegreeofhemosiderindeposition,

tumorsmaydemonstratelowsignal intensityonT1-,T2-,

andprotondensity-weightedsequences;thelowsignalinten-

sityistheresultofthepresenceofhemosiderindeposition.

Hemosiderin typicallydecreasedsignal intensityonlong-

repetitiontimeandlong-echotimespin-echo(SE)sequences

largelyduetotheferromagneticpropertiesofhemosiderin.

OnT2-weightedSEandgradient-echo(GRE)images, the

abnormal tissuemayappear largerbecauseof so-called

blooming.Ontheotherhand,theaccumulationoflipidsin

foamymacrophagesmayproduceareasofhighsignalinten-

sityonT1-weightedSEimagessimilartosubcutaneousfat.

Additionally,areasofhighsignalintensityonT2-weighted

imagescorrespondtoaloculatedcystofjointfluid.Another

well-recognizedcharacteristicsofPVNSonMRimagesare

hyperplasticsynoviumandboneerosioninthejointwithin-

sufficientintra-articularspaceforoutwardproliferationofthe

synovium.Inthispatient,the3.0×2.5cmirregularlyshaped

masshadheterogeneousT2signalintensityandisosignalin-

tensityonT1-weightedMRI.Peripherallowsignalintensity

areasarenotedonbothT1-andT2-weightedMRimages.

(Figs.3-5,10-12)Inthepresentcase, lowsignal intensity

onT1-weightedsequenceswasalsoobserved(Figs.4,11),

butthecausesofheterogeneoushighsignalintensityonT2-

weightedandfatsuppressionT1MRimagesonthemass

werethoughttobeduetotheprofoundfluidfromthecystic

lesionfromthecondyletothesynovium.(Figs.3,5,10,12)

TreatmentofPVNSisexclusivelysurgicalandconsistsof

completeexcisionoftheinvolvedboneandtotalsynovec-

tomybecausesynoviallesionshaveahighrateoflocalrecur-

rence.TherecurrenceofTMJPVNSoccursprimarilyinthe

diffusetypein8%to46%ofcasesandusuallyresultsfrom

incompleteresection,butcanalsobespontaneous14.Radia-

tiontherapymayproduceinvolutionofthelesionbutshould

bereservedforonlythoserecurrentcaseswithinvolvement

ofvitalstructures15.

than80%ofPVNScasesinvolvethekneesandtheupper

extremitiesareseldominvolved,suggestingapredilectionfor

weight-bearingjoints.Eighty-eightpercentageoflocalized

PVNSand59%ofdiffusePVNShaveapositivetraumatic

history.Bloodyfluidwasobtainedinmostofthecasesun-

dergoingsynovialfluidaspiration,andyellowfluidwasob-

tainedinafewcases7,8.

ReportsofPVNSintheTMJarerare,butPVNScanoccur

atanyage,withapreviousincidencepeakinthefourthand

fifthdecadesoflife.Nogenderdifferenceisseen9.Whenthe

diseasedoesoccurintheTMJ,itusuallyarisesasapreau-

ricularpalpablemassorswelling,andisfrequentlymistaken

foraparotidglandlesion.Othersymptomsmayincludeare-

strictedmandibularrangeofmotion,stiffness,numbness,and

pain.Inmostcases,thepainwasofminimalintensity.Are-

viewoftheliteratureandcarefulanalysisoftheclinicopatho-

logicfeaturesofPVNSoftheTMJrevealthat it typically

occursinthediffuseform,andthevastmajorityofreported

caseswereoftheextra-articularvariantofthisdisease,which

isassociatedwithmoreaggressivelocalinfiltrativebehavior,

suchasinvolvingtheparotidglandorskullbase,andahigher

rateoflocalrecurrencethanthelocalizedtype6,10,11.Thepa-

tientinthisstudywasthoughttohavethediffuseform,and

anaggressivelocalrecurrencewithskullbaseperforationby

bonyerosionsparingthedurawasfound4yearsaftertheop-

eration.

TheetiologyofPVNSstillremainscontroversial.While

Jaffeetal.1originally thought itwasaninflammatoryre-

sponsetoanunknownstimulus,othershaveattributedthe

process torepetitive intra-articularhemorrhagefollowing

trauma,disturbancesinlipidmetabolism,orabenignneo-

plasticproliferation7,9.Thepatientinthisstudydidnotrecall

ahistoryoffacialtrauma.

ThemacroscopicfeaturesofPVNSincludeathicksynovi-

umcomprisedofmattedmassesofvilliandsynovialfolds,

orpedunculatednodules.The lesionhasacharacteristic

rusty,red-browncolorresultingfromextensivehemosiderin

deposition.ThemicroscopicfeaturesofPVNSindicatea

nonspecificinflammatoryprocess,resultinginapapillary,

villous,andnodularappearanceofthestratifiedsynovium

withmultinucleatedgiantcells,foamcells,andhemosiderin

deposition3,9.

TheclassicplainradiographicfindingsofPVNSconsistof

cysticerosions,increaseddensityofthesynoviumsecond-

arytohemosiderindeposition,andperiarticularsoft-tissue

swellingwithnocalcification5,9.CTclearlyrevealsareasof

lyticboneerosionandsclerosis.CTalsodefinestheextentof

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146

Conflict of Interest

Nopotentialconflictofinterestrelevanttothisarticlewas

reported.

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