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1、PigmentedVillonodularSynovitis(PVNS)色素沉着绒毛结节性滑膜炎IntroductionPigmentedvillonodularsynovitis(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,usuallymo
2、noarticulardisorderandusuallyfoundinadults.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)ClinicalmanifestationsTheclinicalmanifestationsislackofspecificity.Themainmanifestationsarepro
3、gressiveswellingofjointsandhemorrhagicjointeffusion.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。)ThecauseofPVNSisno
4、tclear,mayberelatedwithtumor、traumaandinfection.(本病的病因尚不明,一般认为与肿瘤、外伤、感染有关。)Diagnosis:imagingfindingsX-ray:theroutineX-rayshowsmajorsymptomsincludingjointcapsuleswelling,softtissuemassinperipheryofjointandinvasionofbonewhichedgeshowsossifiedring,butjointspaceisnormal,noo
5、steoporosis.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:imagingfindingsCTfindings: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:imagingfindingsMRfindingsisveryspecialandshowsthicke
6、nedsynovitisandtheconditionofjointeffusion.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
7、(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。Diffus
8、etypeshowsexuberantsynovial,villiformproliferationandhemosiderindeposition.Exuberantvilluscandestroyjointcapsu