腹膜后纤维化

腹膜后纤维化腹组读片刘婧2010-6-23Introduction腹膜后纤维化retroperitonealfibrosis（RPF）少见的纤维化反应（胶原血管病）1905，由法国泌尿科医生Albarran首次介绍腹膜后纤维化反应继发输尿管狭窄1948，Ormond首次提出RPF的概念慢性主动脉周炎、输尿管周炎、硬化性腹膜后肉芽肿因输尿管梗阻是常见的后遗症，排泄性尿路造影、逆行性肾盂造影可用于发现梗阻的部位及程度CT、MR成为诊断该病及评价继发表现的主力18F-FDGPET核素显像Introduction恶性腹膜后纤维化占8%，预后差熟悉其潜在的临床表现以及典型或不典型的影像学特征是关键Epidemiology发病率为1/200，000好发年龄：40-60；男女比例（2-3）：1绝大部分病例（70%）为特发性腹膜后纤维化其余常与感染、恶性肿瘤以及药物介导伴发罕有儿童及家族遗传病例报道Epidemiology尿路梗阻性疾病全身系统性疾病常与各种免疫紊乱伴发以及对免疫抑制剂有效自身免疫病因假说腹膜后纤维化是对进展期动脉粥样硬化的严重炎症反应假定的过敏原为粥样斑块产生的腊样色素与腹膜后纤维化相关的免疫疾病原发性胆管硬化纤维化纵膈炎肾小球肾炎风湿性关节炎系统性红斑狼疮结节性动脉周围炎强直性脊柱炎桥本甲状腺炎全垂体机能减退硬化性肠系膜炎Clinicalpresentation早期症状腹部或腰部不适下肢水肿或不适（淋巴管闭塞）晚期症状深静脉血栓少尿、无尿恶心、呕吐、尿毒症表现高血压肠系膜缺血肠梗阻恶性淋巴瘤（Hodgkin’s淋巴瘤）转移（结肠、乳腺、肺、泌尿系统、甲状腺原发瘤）腹膜后肉瘤类癌出血主动脉瘤动脉周围血肿创伤或手术炎症Crohn’s病放化疗后感染结核、尿路感染肾脏创伤Pathology大体苍白、血栓样肿块；边界不清；累及邻近器官，如输尿管和下腔静脉镜下纤维增生伴多种炎细胞浸润，如淋巴细胞、巨噬细胞及血管内皮细胞ImagefeaturesConventionalradiographyExcretoryurographysonographyCTMRIScintigraphySonography低回声或无回声、边界清楚、形态不规则腹膜后肿块可伴肾积水、输尿管积水尾侧超出骶骨岬，且不呈分叶状----良性敏感性低，25%原发性胆管硬化胆管扩张（胆总管狭窄）门脉高压（门静脉受压）胰管局部或弥漫扩张（硬化性胰腺炎）Sonography60-year-oldmanwithbiopsy-provenidiopathicretroperitonealfibrosis.A,Transversesonogramatlevelofmidaortarevealspresenceofparaaorticandpreaortichypoechoicsofttissuemass(arrows).Rightureteralandpelvicalycealdilatationwerefoundtocoexist.B,CorrelatingCTimagealsoshowsobstructiveuropathy(arrowheads)resultingfromureteralinvolvementthatprecludedcontrastadministration.Notethatcalcifiedabdominalaortaisnotelevatedfromunderlyinglumbarspineandrelativelysmoothperipheralmarginsofabnormalsofttissue(arrows).CT病变定位、范围、邻近器官及血管有利于显示病因腹主动脉瘤胰腺炎、肠系膜腺病腹膜后肿块、位于脊柱旁、边界清楚、形态不规则、呈等密度CT腹主动脉或髂动脉周围输尿管后腹膜腹主动脉分叉处中心向前十二指肠胰腺脾脏纵隔骶骨头尾侧一般不会发生骨破坏，但恶性疾病可继发一般良性病变中腹主动脉和下腔静脉不会发生移位，但也有例外CT强化强化程度与纤维化进展相关显著强化---急性期；低强化或无强化---进展期或慢性疾病Brun等发现约1/3经手术病理证实的RPF患者无CT异常表现CT55-year-oldmanwithretroperitonealfibrosis.AandB,AxialoralandIVcontrast-enhancedCTimagesshowpresenceoflow-attenuationmassanteriorandlateraltoaortaandiliacvessels,withoutanteriordisplacementofeitheraortaorinferiorvenacava.Retroperitonealmassobliteratesfatplanebetweenvesselsandpsoasmuscle(arrows,A).Plaquebifurcatesandfollowscommoniliacarteries(arrowhead,B).CT55-year-oldmanwithinflammatoryabdominalaorticaneurysm.AandB,OralandIVcontrast-enhancedaxial(A)andcoronal(B)CTimagesshowill-definedmassofsoft-tissueattenuationsurroundingatheromatousaneurysm.Bilateralnephrostomytubeshavebeenplacedforobstructiveuropathy.CT在鉴别良恶性病变中的应用比较困难恶性征象体积巨大，占位效应，主动脉及下腔静脉移位（原因可能是血管后方的淋巴结肿大）易形成结节，呈分叶状良性征象“tethering”血栓密度，向周围浸润、蔓延但是敏感性和特异性都较低CT在鉴别良恶性病变中的应用50-year-oldmanwithbiopsy-confirmednon-Hodgkin’slymphoma.AandB,Contrast-enhancedCTscansshowbulkysoft-tissuemass(arrow,A)surroundingaortaandinferiorvenacava.Noteslightelevationofaortafromspine,featuresuggestiveofneoplasia.CT在鉴别良恶性病变中的应用64-year-oldwomanwithabdominalpain.Contrast-enhancedabdominalCTscanrevealspresenceofretroperitonealmass.Aortaisminimallyelevatedfromunderlyingspine,raisingconcernforunderlyingneoplasia.CT-guidedbiopsyandsubsequentcystoscopyconfirmedpresenceofmetastatictransitionalcellcarcinomaofurinarybladder.CT在鉴别良恶性病变中的应用35-year-oldmanwithHIVwhopresentedwithabdominalpainandfever.Sputumcultureandchestradiographysuggestedtuberculosis.IVcontrast-enhancedCTscanofabdomenshowsnonlobulatedretroperitonealparaaorticmassofsofttissueattenuation.Biopsyconfirmedbenigninfectivelymphadenopathy.CT在鉴别良恶性病变中的应用35-year-oldwomanwithendometrialadenocarcinoma.Confluentlow-attenuationretroperitonealmetastaticdeposits(arrow)haveappearancesimilartothatofretroperitonealfibrosis.CTscanshowsthissofttissueiscenteredonlowerinfrarenalaorta,hasrelativelysmoothmargins,anddoesnotelevateaortafromspine—featuresthatmayallowdifferentiationofmalignantfrombenignretroperitonealfibrosis.Noteassociatedlefthydronephrosis(arrowhead).CT在鉴别良恶性病变中的应用47year-oldmanshowsretroperitonealmassthatfailstoelevateaortafromspine.However,thismasshassuspiciouslobulatedanteriormargin.Uppergastrointestinalendoscopy(notshown)revealedpresenceofgastricadenocarcinoma,withsubsequentbiopsy-provenretroperitonealmetastasis.CT在鉴别良恶性病变中的应用50-year-oldwomanwithnewdiagnosisofpancreaticadenocarcinoma.CTscanshowsparaaorticretroperitonealsoft-tissuemassbutnoelevationofaortafromspine,whichsuggestsbenigncause.However,thismasshaslobulatedanteriormargin,whichraisesconcernformetastaticdisease.Subsequentbiopsyconfirmedmalignantnatureofthisparaaorticsofttissue.MRI腹膜后组织结构显影、病因以及并发症的显示信号特征与其他纤维变性相似弥漫T1WI低信号增强可反映水肿程度慢性、非活动期的纤维组织T1、T2WI均低信号可用于评价患者对治疗的反应----水肿减轻，提示疗效好强化程度减轻也提示好转MRI50-year-oldmanwithretroperitonealfibrosis.AandB,Inflammatoryabdominalaneurysmandinflammatoryretroperitonealfibrosisareseenonfat-saturatedaxialT1gradient-recalledechoimage(A).MRimagesshownear-circumferentialparaaorticsoft-tissuemasswithoutelevationofaortafromunderlyingspine.Righthydronephrosisandrightrenalatrophyhaveresulted.Contrast-enhancedimage(B)showsintenseenhancementofretroperitonealfibrosis,consistentwithactiveinflammation.MRI60-year-oldmanwithidiopathicretroperit