色素沉着绒毛结节性滑膜炎的MRI表现ppt课件

.,PigmentedVillonodularSynovitis(PVNS)色素沉着绒毛结节性滑膜炎,.,Introduction,Pigmentedvillonodularsynovitis(PVNS)comprisesagroupofidiopathiclesionsinjoints,tendonsheathesandbursae.(色素沉着绒毛结节性滑膜炎主要是指关节、滑囊以及腱鞘内的滑膜特发性的呈结节状或绒毛状进行性增生。)Pigmentedvilionodularsynovitis(PVNS)isanuncommon,usuallymonoarticulardisorderandusuallyfoundinadults.Themostcommonsitespredilectionisknee-joint(80%),thenthehip,ankle,shoulder,elbowinturn.(不常见，好发于成年人，生于膝关节,占80%,髋关节、踝关节、肩关节、肘关节的发病率依次减少),.,Clinicalmanifestations,Theclinicalmanifestationsislackofspecificity.Themainmanifestationsareprogressiveswellingofjointsandhemorrhagicjointeffusion.Itshowsrecurrentjointeffusions,whichcanbemisdiagnosedasarthritis.Episodesofcompleteremissionmaybefoundbetweenperiodsofactivedisease.(本病的临床表现缺乏特异性.临床主要表现为受累关节进行性肿胀,血性关节积液较常见，本病可引起反复积液，各活动期之间可见病变发作的缓解。)ThecauseofPVNSisnotclear,mayberelatedwithtumor、traumaandinfection.(本病的病因尚不明,一般认为与肿瘤、外伤、感染有关。),.,Diagnosis：imagingfindings,X-ray:theroutineX-rayshowsmajorsymptomsincludingjointcapsuleswelling,softtissuemassinperipheryofjointandinvasionofbonewhichedgeshowsossifiedring,butjointspaceisnormal,noosteoporosis.X线表现:常规X线平片上主要征象包括关节囊肿胀、关节周围软组织肿块及邻近骨骼的侵蚀,骨缺损边缘有硬化环,但关节间隙保持正常,无骨质疏松,.,Diagnosis：imagingfindings,CTfindings:showsjointcapsuleswelling,thickenedsynoviumandobviousjointeffusionCT表现:表现为关节囊肿胀,滑膜增厚,关节腔内明显积液,.,Diagnosis：imagingfindings,MRfindingsisveryspecialandshowsthickenedsynovitisandtheconditionofjointeffusion.MRI表现具有明显的特征性，能清晰的显示滑膜的增厚和积液的程度。Becauseofhemosiderindepositioninthesynovitislesions,itshowslowsignalbothonT1WIandT2WI.Partiallesionsshowboneinvasion.由于病变滑膜组织内含铁血黄素的沉积，故在T1加权像和T2加权像均呈低信号，这是特征性的征象。部分病变可侵蚀骨结构。,.,HistologyandPathology(组织病理学),Thehistologicalfindingsareexuberantsynovialproliferationwithnumerousvilliandfoldsthatsometimesfuseintonodulesandformlocallyaggressiveintra-articularmasses.组织学表现为活跃增生的滑膜呈绒毛状或皱襞样，常形成结节状侵袭性的关节内肿物。PVNScanbedividedintofocaltypeanddiffusetype.PVNS在病理上分为局灶型和弥漫型2种。,.,Diffusetypeshowsexuberantsynovial,villiformproliferationandhemosiderindeposition.Exuberantvilluscandestroyjointcapsuleandsofttissue,andinvadebonebygettingthrougharticularcartilage,junctionofboneandjointortheattachmentofligment.弥漫型主要为滑膜广泛增厚、绒毛状增生和含铁血黄素沉着,增殖的绒毛可破坏关节囊,侵犯周边软组织并通过关节软骨、骨与关节交界部或沿韧带附着处侵犯骨组织Focaltypeshowsmoundsofsynovialcells,dispersedmultinucleatedgiantcells,foamcellsandpigmentedhemosiderindeposition.局灶型为密集成堆的滑膜细胞,间以散在的多核巨细胞和有类脂质积聚的泡沫细胞,以及含铁血黄素沉着。,.,Diffusetype：T2WIshowsmultiplelowsignalnodulesinjointandpoplitealspace,highsignalalsocanbeseeninpartialnoduleswithjointeffusionshowshyperintense.Articularsurfaceoftibialplatformwasinvadedandshowmixedsignalsurroundingbylowsignalring.Lowsignalnodulealsocanbeseeninthesuprapatellarbursa.弥漫型PVNS:T2WI示关节腔内及腘窝多发低信号结节,部分结节内见高信号区,关节腔积液呈高信号。胫骨关节面破坏,呈混杂信号,周围伴低信号环。髌上囊亦见低信号结节.Focaltype：T1WIshowswell-distributedlowsignalmassininfrapatellarbursawithalittleeffusionandjointstructuresisnormal.局灶型PVNS,T1WI示髌下囊内肿块,肿块呈均匀低信号,伴关节腔少量积液,关节骨结构正常,.,Case135-year-oldfemalepatient，progressiveswellingofkneejointsfor5years女，35岁，膝关节进行性肿胀5年,Diffusetypeofkneejoint:T1WIshowslowsignalirregularthickenedsynoviumontheanteriorandtheposteriorcruciateligament.膝关节弥漫型PVNS。MRT1WI示前、后交叉韧带表面有不规则增厚的滑膜覆盖,增厚的滑膜呈低信号,.,T2WIalsoshowslowsignalirregularthickenedsynoviumontheanteriorandtheposteriorcruciateligament.Lowsignalnodulescanbeseenintheeffusionofsuprapatellarbursa.T2WIshowsmixedsignalofmultiplebonedestruction,surroundingbyarimofhypointensity.T2WI示前、后交叉韧带表面有不规则增厚的低信号滑膜覆盖,髌上囊积液内见低信号结节(图5)。T2WI示股骨、胫骨多发骨破坏,破坏区高低信号混杂,伴周边低信号环(图6),.,Case240-year-oldfemalepatient,diffusetypePVNS,Figure4:T1WIshowsdiffusivethickenedsynovium.Suprapatellarbursabecomelargebecauseofcloddysynovium.Infrapatellarfatpaddisappearinsteadofproliferoussynovium,anteriorhornofthelateralmeniscuswasinvolved.Figure5:T2WI:diffusiveproliferoussynoviumshowslowsignalbecauseofhemosiderindeposition.MRIT1加权像,滑膜弥漫性增厚,髌上囊因被团状的滑膜组织占据而扩大,髌下脂肪垫内脂肪缺失,代之以增厚的滑膜组织,外侧前角半月板受累及。图5MRI示T2加权像,弥漫增厚的滑膜呈低信号,系含铁血黄素沉积所致。,.,MRIshowsnoduleonthesurfaceofanteriorcruciateligamentandbubblelowsignalunderarticularsurfaceoftibialplatform.Histopathologicsectionshowsproliferoussynovialcell,interstitial(withintissues)showroundnessandpolygon.Partialcellshavehemosiderindeposition,multinucleatedgiantcellscanbeseenlocally.MRI示矢状面T1、T2加权像,前十字韧带表面结节影,胫骨关节面下类圆形低信号骨质缺损。HE400滑膜细胞明显增生,间质浸润的组织呈圆形或多角形,部分细胞吞噬含铁血黄素,局部见融合的多核巨细胞。,.,Case326-year-oldmalepatient,diffusetypeofrightkneejoint男,26岁,右膝关节弥漫色素沉着绒毛结节性滑膜炎,Figure1.T1WI:stratifiedsingularsignalinthepoplitealfossashowsslightlyhighcentreandiso-lowrim.Irregularbandedlowsignalcanbeseeninthesuprapatellarbursaandinfrapatellarfatpad.Obviouslylowsignalcanbeseenbelowthearticularsurfaceoftibia.图1右膝关节矢位T1WI示腘窝内见异常信号,呈中心稍高,周边等、低信号的分层状排列,髌上囊及髌下脂肪囊内见形态不规则的片带状低信号影,胫骨前上缘关节面下见小囊状明显低信号影。Figure2.fat-suppressedT2WI:stratifiedsingularsignalinthepoplitealfossashowsobviouslyhighcentreandiso-lowrim.Irregularbandedhighsignalcanbeseeninthesuprapatellarbursaandinfrapatellarfatpad.Mixedsignalcanbeseenbelowthearticularsurfaceoftibia.图2右膝关节矢位压脂T2WI示腘窝内异常信号,呈中心明显高、周边等、低信号的分层状排列,髌上囊及髌下脂肪囊内见形态不规则的片带状高信号影,胫骨前上缘关节面下见小囊状高、低混杂信号影。,.,figure3.fat-suppressedT1WIaftercontrastenhanced:thecenterofthelesioninthepoplitealspaceshowsmoderateenhanced,therimofthelesion、suprapatellarbursa、infrapatellarfatpadandpoplitealspacewereobviouslyenhanced.Obviouslyannularenhancedlessionalsobeseenbelowthearticularsurfaceoftibia压脂T1WI增强扫描示腘窝内病灶,中心部分呈中度增强,周边及髌上囊及髌下脂肪囊、腘窝内见形态不规则的片带状明显增强,胫骨前上缘关节面下见环状明显增强。,.,Case422-year-oldmalepatient,thePVNSoflefthipjoint男性,22岁,右髋关节色素沉着绒毛结节性滑膜炎,Thenodularlesionofrighthipjointshowshyper-hypointense-signalonT2WIandiso/hypomixed-signalonT1WI.TherightfemoralheadwascompressivedeformationandshowmultiplenodularlowsignalonT2WIandT1WI.右髋关节轴位T2WI示右髋关节内见结节状高、低混杂信号,相应区域股骨颈受压变形,股骨头内见多发结节状低信号影.轴位T1WI示右髋关节内见结节状等低混杂信号相应区域股骨颈受压变形,股骨头内见多发结节状低信号影.,.,Figure6.enhancedT1WI:thenodularlesionoftherighthipjointshowsobviouslyenhanced.轴位T1WI增强扫描示右髋关节内结节状异常信号明显增强。,.,Case5A43-year-oldmanpresentedwithpaininthebackoftheneckradiatingtotherightupperlimb.Thepainhadprogressedoveraperiodof3months.Therewasnoweaknessofthelowerlimbsorincontinenceofbladderorbowel.43岁男性，颈背部疼痛并放射至右上肢，进行性加重3个月，下肢无症状，无大小便失禁,FIG1.Lateralradiographofthecervicalspineshowsasofttissuemassdestroyingtheposteriorelementsofthelowercervicalspine(arrow).Therearenofociofcalcificationsseenwithinthismass.图1：颈椎侧位片示软组织肿块破坏颈椎后下部结构，肿块内无钙化点。,.,FIG2.SagittalT1-weightedimagesbefore(A)andafter(B)contrastdministrationshowanexpansilemasslesioninvolvingtheposteriorelementsofC5andC6vertebra.Thismassshowshomogeneousenhancementaftercontrastmaterialadministration.Itisseentodisplacethethecalsacanteriorly.图2.矢状位T1WI增强前后示膨胀性的肿块侵犯惊5、6椎体及其附件，肿块呈均匀强化，向前推压硬膜囊。,.,AxialT1-weightedpostcontrastimagewithfatsuppressionshowstheexactdelineationofthemasswithananteriorlydisplacedthecalsac(arrow).轴位增强压脂T1WI示界限明确的肿块向前推压硬膜囊。Histopathologicsectionofpigmentedvillonodularsynovitisdepictingsheetlikegrowthwithinthemaintumormass,whichiscomprisedofmononuclearcellsandmultinucleatedgiantcellswithinthecollagenizedstroma.Hematoxylinandeosin.组织病理学示变形增