色素沉著絨毛結(jié)節(jié)性滑膜炎的診療進展

Progressinthediagnosisandtreatmentofpigmentedvillonodularsynovitis

色素沉著絨毛結(jié)節(jié)性滑膜炎（PVNS）的診治進展

PVNSisararediseasethatoccursinsynovialtissue-likehyperplasia.色素沉著絨毛結(jié)節(jié)性滑膜炎是一種罕見的發(fā)生于滑膜組織瘤樣增生疾病。基本概念

病因與發(fā)病機制Theetiologyandpathogenesisarestillunclear.

PVNS病因及發(fā)病機制至今仍不清楚

Mayberelatedtotraumaandbleeding,lipidmetabolismdisorders,chronicinflammatoryreactions,tumors,etc.

可能的病因主要包括：創(chuàng)傷及出血、脂質(zhì)代謝紊亂、慢性炎癥反應(yīng)、腫瘤等

目前認為創(chuàng)傷及出血可能加重PVNS病情而引發(fā)癥狀，其并非誘發(fā)該疾病的必要因素。發(fā)病特點Thediseaseoccursinyoungadults.

該疾病好發(fā)于青壯年，男女發(fā)病率無明顯差異。Chroniccourse.

慢性病程，多為1-4年，可長達10年。Mostlysinglejointdisease.多為單關(guān)節(jié)發(fā)病，occur:knee>hip>ankle>elbow.好發(fā)部位：膝＞髖＞踝＞肘。分型LPVNS局限性PVNSDPVNS彌散性PVNSAccordingtotheextentoflesiondamage,itisdividedintoLPVNSandDPVNS.僅部分關(guān)節(jié)滑膜受累，病灶多為單個帶蒂的黃色或棕黃色結(jié)節(jié)狀突起。關(guān)節(jié)內(nèi)有血性漿液滲出物。手術(shù)后預(yù)后良好表現(xiàn)為彌散性增厚和絨毛狀結(jié)構(gòu)，關(guān)節(jié)韌帶和骨組織侵襲。關(guān)節(jié)液多為深棕色或黃色。手術(shù)難徹底清除病灶，易復發(fā)。癥狀與體征PVNS主要臨床癥狀是：緩慢進行性腫脹、疼痛及功能受限。LPVNS常表現(xiàn)為膝關(guān)節(jié)腫脹，亦可出現(xiàn)膝關(guān)節(jié)絞索。DPVNS因大量積液和滑膜增生，可出現(xiàn)浮髕征陽性，關(guān)節(jié)活動受限，關(guān)節(jié)腔抽吸液呈暗紅色或鐵銹色液體。PVNS臨床表現(xiàn)無特異性，易與其他炎癥性疾病混淆。PVNSsymptom：Slowprogressiveswelling、pain、Limitedfunction。

LPVNSSign：Jointswelling，Kneejointnoose。DPVNSSign：thefloatingtestwaspostive，Limitedjointactivity，Thejointcavityaspirateisdarkred。Auxiliaryexamination:Imagingexamination：X-ray、CT、MRI.Examinationofjointcavityaspirationfluid.Arthroscopicexploration.Pathologicalexamination.影像學檢查：X線片、CT、MRI（最有意義）關(guān)節(jié)腔抽液檢查關(guān)節(jié)鏡探查病理檢查（確診）輔助檢查：Ｘ線檢查

約２０％ＰＶＮＳ患者X線表現(xiàn)正常，缺乏早期診斷價值。最常見的表現(xiàn)是關(guān)節(jié)軟組織腫脹，由于含鐵血黃素的沉著而密度增加，但沒有鈣化。當病變累及軟骨和骨時，可出現(xiàn)邊緣性鋸齒樣骨質(zhì)破壞及大小不等的囊狀骨質(zhì)缺損區(qū)。繼發(fā)骨關(guān)節(jié)炎時，則有關(guān)節(jié)間隙狹窄，關(guān)節(jié)面不平，關(guān)節(jié)內(nèi)游離體。

X-rayfindingswerenormalinabout20%ofPVNSpatients.Themostcommonmanifestationissofttissueswellingofthejoints.Whenlesionsinvolvecartilageandbone,bonedestructionandbonedefectareasoccur.CTACTscancandeterminetheextentofthePVNSlesion.Itcanshowjointfluideffusion,synovialthickeningandsynovialnodulebulging,especiallytheextentofboneinvolvement,butitcannotshowthedestructionofarticularcartilage,norcanitdeterminethenatureofthelesion.CT檢查能確定PVNS病變侵襲范圍。能顯示關(guān)節(jié)囊積液、滑膜增厚和滑膜結(jié)節(jié)突起，尤其是骨質(zhì)受累范圍，但無法顯示關(guān)節(jié)軟骨破壞，不能明確病變性質(zhì)。

MRIiscurrentlythebestdiagnostictool.ThelesionsinthesynoviumshowednonuniformlowsignalonT1andT2.ThelowT2signalwascharacteristicofPVNSandcouldbeusedasanimportantbasisforthediagnosisofPVNS.

MRI是目前最佳的診斷手段?；?nèi)病灶在T1、T2上均表現(xiàn)為不均勻的低信號，T2低信號是PVNS特征性表現(xiàn)，可作為診斷PVNS的重要依據(jù)。MRIThejointcavityaspirateisusuallyyellowishbrownordarkred.

關(guān)節(jié)腔抽液：常為黃褐色或暗紅色。

Intraarticularlesionscanbeclearlyseenbyarthroscopy.

關(guān)節(jié)鏡檢查可清晰看見關(guān)節(jié)內(nèi)病灶關(guān)節(jié)鏡探查：病理檢查（金標準）

PathologicalexaminationisthegoldstandardforthediagnosisofPVNS.

Microscopically,proliferatingsynovialcells,macrophagesandfibroblastswereseen,andcharacteristicfoamcellswereclustered.

鏡下可見增生的滑膜細胞、成纖維細胞、巨噬細胞，且特征性泡沫細胞聚集，細胞外含鐵血黃素沉著。治療CompleteremovalofsynovialtissueisthekeytothetreatmentofPVNS.徹底清除病變的滑膜組織是治療PVNS的關(guān)鍵。Therapeuticmethodsinclude:surgicaltreatment,surgerycombinedwithradiotherapy,biologicaltargetingtherapy.1.手術(shù)：開放性滑膜切除術(shù)

關(guān)節(jié)鏡下滑膜切除手術(shù)關(guān)節(jié)鏡聯(lián)合開放性手術(shù)關(guān)節(jié)置換術(shù)2.手術(shù)聯(lián)合放療3.生物靶向治療LPVNS治療ThelesionsofLPVNSarelocalizedandcanusuallybecompletelyresectedunderarthroscopy.Thetraditionalopensynoviectomyhasbeenreplacedbyarthroscopicsurgery.LPVNS：病變局限，通?？稍陉P(guān)節(jié)鏡下全部切除病變滑膜預(yù)后良好。傳統(tǒng)的開放性滑膜切除術(shù)已逐漸被關(guān)節(jié)鏡手術(shù)所替代。DPVNS手術(shù)治療1.Opensurgery:therearedisadvantagessuchaslargesurgicaltrauma,easystiffnessofjoints,longhospitalstayandlongrecoveryperiod.開放性手術(shù)：存在手術(shù)創(chuàng)傷大、關(guān)節(jié)易僵硬、住院時間長、恢復期長等缺點。2.Arthroscopicsurgery:ForDPVNSwithlongcourseandwideinvasiverange,arthroscopicsurgeryaloneisdifficulttocompletelyremovetheaffectedsynovium,andthereisahighriskofrecurrence.關(guān)節(jié)鏡手術(shù)：對于病程長、病變侵襲范圍廣的DPVNS，單純關(guān)節(jié)鏡手術(shù)很難完全切除病變滑膜，存在高復發(fā)風險。3.Arthroscopycombinedwithopensurgery:somescholarsproposedthatforDPVNS,therecurrencerateofarthroscopycombinedwithopensurgerywassignificantlylowerthanthatofpurearthroscopy.關(guān)節(jié)鏡聯(lián)合開放性手術(shù)：有學者提出，對于ＤＰＶＮＳ，關(guān)節(jié)鏡聯(lián)合開放性手術(shù)術(shù)后復發(fā)率明顯低于單純關(guān)節(jié)鏡手術(shù)。DPVNS手術(shù)治療4.TKA:ForadvancedPVNSwithseverebonedestructionorosteoarthritis,TKAaftersynovectomyisdesirable.關(guān)節(jié)置換：晚期PVNS,對于嚴重骨質(zhì)破壞或伴發(fā)骨性關(guān)節(jié)炎的PVNS,在滑膜切除后需行TKA。Atpresent,onlyopensurgeryhasbeenlessusedtoresectthesynoviumofthelesion.Theefficacyofarthroscopicsurgeryhasbeenrecognizedbymostscholars,whichmakesupfortheshortcomingsofopensurgeryandimprovesthecurerateandcomplicationrateofDPVNS.目前單純開放性手術(shù)切除病變滑膜已較少應(yīng)用，關(guān)節(jié)鏡手術(shù)療效得到多數(shù)學者認可，彌補了開放性手術(shù)的不足，使DPVNS治愈率和并發(fā)癥發(fā)生率明顯改善。手術(shù)聯(lián)合放療

BecauseDPVNSischaracterizedbyextensiveinvasion,growthandevenmalignancy,itisverydifficulttoremovesynoviumafterarthroscopicsurgeryoropensurgery.Thereisacertainrecurrencerateaftersurgery.RadiotherapyisoftenusedasadjuvanttreatmentafterDPVNS.由于DPVNS有廣泛侵襲生長甚至惡變的特點，無論是關(guān)節(jié)鏡手術(shù)還是開放性手術(shù)都很難將滑膜切除干凈,術(shù)后均有一定的復