色素沉着绒毛结节性滑膜炎的诊疗进展

Progressinthediagnosisandtreatmentofpigmentedvillonodularsynovitis

色素沉着绒毛结节性滑膜炎（PVNS）的诊治进展

PVNSisararediseasethatoccursinsynovialtissue-likehyperplasia.色素沉着绒毛结节性滑膜炎是一种罕见的发生于滑膜组织瘤样增生疾病。基本概念

病因与发病机制Theetiologyandpathogenesisarestillunclear.

PVNS病因及发病机制至今仍不清楚

Mayberelatedtotraumaandbleeding,lipidmetabolismdisorders,chronicinflammatoryreactions,tumors,etc.

可能的病因主要包括：创伤及出血、脂质代谢紊乱、慢性炎症反应、肿瘤等

目前认为创伤及出血可能加重PVNS病情而引发症状，其并非诱发该疾病的必要因素。发病特点Thediseaseoccursinyoungadults.

该疾病好发于青壮年，男女发病率无明显差异。Chroniccourse.

慢性病程，多为1-4年，可长达10年。Mostlysinglejointdisease.多为单关节发病，occur:knee>hip>ankle>elbow.好发部位：膝＞髋＞踝＞肘。分型LPVNS局限性PVNSDPVNS弥散性PVNSAccordingtotheextentoflesiondamage,itisdividedintoLPVNSandDPVNS.仅部分关节滑膜受累，病灶多为单个带蒂的黄色或棕黄色结节状突起。关节内有血性浆液渗出物。手术后预后良好表现为弥散性增厚和绒毛状结构，关节韧带和骨组织侵袭。关节液多为深棕色或黄色。手术难彻底清除病灶，易复发。症状与体征PVNS主要临床症状是：缓慢进行性肿胀、疼痛及功能受限。LPVNS常表现为膝关节肿胀，亦可出现膝关节绞索。DPVNS因大量积液和滑膜增生，可出现浮髌征阳性，关节活动受限，关节腔抽吸液呈暗红色或铁锈色液体。PVNS临床表现无特异性，易与其他炎症性疾病混淆。PVNSsymptom：Slowprogressiveswelling、pain、Limitedfunction。

LPVNSSign：Jointswelling，Kneejointnoose。DPVNSSign：thefloatingtestwaspostive，Limitedjointactivity，Thejointcavityaspirateisdarkred。Auxiliaryexamination:Imagingexamination：X-ray、CT、MRI.Examinationofjointcavityaspirationfluid.Arthroscopicexploration.Pathologicalexamination.影像学检查：X线片、CT、MRI（最有意义）关节腔抽液检查关节镜探查病理检查（确诊）辅助检查：Ｘ线检查

约２０％ＰＶＮＳ患者X线表现正常，缺乏早期诊断价值。最常见的表现是关节软组织肿胀，由于含铁血黄素的沉着而密度增加，但没有钙化。当病变累及软骨和骨时，可出现边缘性锯齿样骨质破坏及大小不等的囊状骨质缺损区。继发骨关节炎时，则有关节间隙狭窄，关节面不平，关节内游离体。

X-rayfindingswerenormalinabout20%ofPVNSpatients.Themostcommonmanifestationissofttissueswellingofthejoints.Whenlesionsinvolvecartilageandbone,bonedestructionandbonedefectareasoccur.CTACTscancandeterminetheextentofthePVNSlesion.Itcanshowjointfluideffusion,synovialthickeningandsynovialnodulebulging,especiallytheextentofboneinvolvement,butitcannotshowthedestructionofarticularcartilage,norcanitdeterminethenatureofthelesion.CT检查能确定PVNS病变侵袭范围。能显示关节囊积液、滑膜增厚和滑膜结节突起，尤其是骨质受累范围，但无法显示关节软骨破坏，不能明确病变性质。

MRIiscurrentlythebestdiagnostictool.ThelesionsinthesynoviumshowednonuniformlowsignalonT1andT2.ThelowT2signalwascharacteristicofPVNSandcouldbeusedasanimportantbasisforthediagnosisofPVNS.

MRI是目前最佳的诊断手段。滑膜内病灶在T1、T2上均表现为不均匀的低信号，T2低信号是PVNS特征性表现，可作为诊断PVNS的重要依据。MRIThejointcavityaspirateisusuallyyellowishbrownordarkred.

关节腔抽液：常为黄褐色或暗红色。

Intraarticularlesionscanbeclearlyseenbyarthroscopy.

关节镜检查可清晰看见关节内病灶关节镜探查：病理检查（金标准）

PathologicalexaminationisthegoldstandardforthediagnosisofPVNS.

Microscopically,proliferatingsynovialcells,macrophagesandfibroblastswereseen,andcharacteristicfoamcellswereclustered.

镜下可见增生的滑膜细胞、成纤维细胞、巨噬细胞，且特征性泡沫细胞聚集，细胞外含铁血黄素沉着。治疗CompleteremovalofsynovialtissueisthekeytothetreatmentofPVNS.彻底清除病变的滑膜组织是治疗PVNS的关键。Therapeuticmethodsinclude:surgicaltreatment,surgerycombinedwithradiotherapy,biologicaltargetingtherapy.1.手术：开放性滑膜切除术

关节镜下滑膜切除手术关节镜联合开放性手术关节置换术2.手术联合放疗3.生物靶向治疗LPVNS治疗ThelesionsofLPVNSarelocalizedandcanusuallybecompletelyresectedunderarthroscopy.Thetraditionalopensynoviectomyhasbeenreplacedbyarthroscopicsurgery.LPVNS：病变局限，通常可在关节镜下全部切除病变滑膜预后良好。传统的开放性滑膜切除术已逐渐被关节镜手术所替代。DPVNS手术治疗1.Opensurgery:therearedisadvantagessuchaslargesurgicaltrauma,easystiffnessofjoints,longhospitalstayandlongrecoveryperiod.开放性手术：存在手术创伤大、关节易僵硬、住院时间长、恢复期长等缺点。2.Arthroscopicsurgery:ForDPVNSwithlongcourseandwideinvasiverange,arthroscopicsurgeryaloneisdifficulttocompletelyremovetheaffectedsynovium,andthereisahighriskofrecurrence.关节镜手术：对于病程长、病变侵袭范围广的DPVNS，单纯关节镜手术很难完全切除病变滑膜，存在高复发风险。3.Arthroscopycombinedwithopensurgery:somescholarsproposedthatforDPVNS,therecurrencerateofarthroscopycombinedwithopensurgerywassignificantlylowerthanthatofpurearthroscopy.关节镜联合开放性手术：有学者提出，对于ＤＰＶＮＳ，关节镜联合开放性手术术后复发率明显低于单纯关节镜手术。DPVNS手术治疗4.TKA:ForadvancedPVNSwithseverebonedestructionorosteoarthritis,TKAaftersynovectomyisdesirable.关节置换：晚期PVNS,对于严重骨质破坏或伴发骨性关节炎的PVNS,在滑膜切除后需行TKA。Atpresent,onlyopensurgeryhasbeenlessusedtoresectthesynoviumofthelesion.Theefficacyofarthroscopicsurgeryhasbeenrecognizedbymostscholars,whichmakesupfortheshortcomingsofopensurgeryandimprovesthecurerateandcomplicationrateofDPVNS.目前单纯开放性手术切除病变滑膜已较少应用，关节镜手术疗效得到多数学者认可，弥补了开放性手术的不足，使DPVNS治愈率和并发症发生率明显改善。手术联合放疗

BecauseDPVNSischaracterizedbyextensiveinvasion,growthandevenmalignancy,itisverydifficulttoremovesynoviumafterarthroscopicsurgeryoropensurgery.Thereisacertainrecurrencerateaftersurgery.RadiotherapyisoftenusedasadjuvanttreatmentafterDPVNS.由于DPVNS有广泛侵袭生长甚至恶变的特点，无论是关节镜手术还是开放性手术都很难将滑膜切除干净,术后均有一定的复