【教學(xué)課件】腹膜后纖維化

1、腹膜后纖維化第1頁，共32頁。Introduction 腹膜后纖維化retroperitoneal fibrosis（RPF）少見的纖維化反應(yīng)（膠原血管?。?905，由法國泌尿科醫(yī)生Albarran首次介紹腹膜后纖維化反應(yīng)繼發(fā)輸尿管狹窄1948，Ormond首次提出RPF的概念慢性主動脈周炎、輸尿管周炎、硬化性腹膜后肉芽腫因輸尿管梗阻是常見的后遺癥，排泄性尿路造影、逆行性腎盂造影可用于發(fā)現(xiàn)梗阻的部位及程度CT、MR成為診斷該病及評價繼發(fā)表現(xiàn)的主力18F-FDG PET核素顯像第2頁，共32頁。Introduction 惡性腹膜后纖維化占8%，預(yù)后差熟悉其潛在的臨床表現(xiàn)以及典型或不典型的影像學(xué)特

2、征是關(guān)鍵第3頁，共32頁。Epidemiology發(fā)病率為1/200，000好發(fā)年齡：40-60；男女比例（2-3）：1絕大部分病例（70%）為特發(fā)性腹膜后纖維化其余常與感染、惡性腫瘤以及藥物介導(dǎo)伴發(fā)罕有兒童及家族遺傳病例報(bào)道第4頁，共32頁。Epidemiology尿路梗阻性疾病 全身系統(tǒng)性疾病常與各種免疫紊亂伴發(fā)以及對免疫抑制劑有效 自身免疫病因假說腹膜后纖維化是對進(jìn)展期動脈粥樣硬化的嚴(yán)重炎癥反應(yīng)假定的過敏原為粥樣斑塊產(chǎn)生的臘樣色素第5頁，共32頁。與腹膜后纖維化相關(guān)的免疫疾病原發(fā)性膽管硬化纖維化縱膈炎腎小球腎炎風(fēng)濕性關(guān)節(jié)炎系統(tǒng)性紅斑狼瘡結(jié)節(jié)性動脈周圍炎強(qiáng)直性脊柱炎橋本甲狀腺炎全垂體機(jī)能減

3、退硬化性腸系膜炎第6頁，共32頁。Clinical presentation早期癥狀腹部或腰部不適下肢水腫或不適（淋巴管閉塞）晚期癥狀深靜脈血栓少尿、無尿惡心、嘔吐、尿毒癥表現(xiàn)高血壓腸系膜缺血腸梗阻第7頁，共32頁。惡性淋巴瘤（Hodgkins淋巴瘤）轉(zhuǎn)移（結(jié)腸、乳腺、肺、泌尿系統(tǒng)、甲狀腺原發(fā)瘤）腹膜后肉瘤類癌出血主動脈瘤動脈周圍血腫創(chuàng)傷或手術(shù)炎癥Crohns病放化療后感染結(jié)核、尿路感染腎臟創(chuàng)傷第8頁，共32頁。Pathology大體蒼白、血栓樣腫塊；邊界不清；累及鄰近器官，如輸尿管和下腔靜脈鏡下纖維增生伴多種炎細(xì)胞浸潤，如淋巴細(xì)胞、巨噬細(xì)胞及血管內(nèi)皮細(xì)胞第9頁，共32頁。Image feat

4、uresConventional radiographyExcretory urographysonographyCTMRIScintigraphy 第10頁，共32頁。Sonography低回聲或無回聲、邊界清楚、形態(tài)不規(guī)則腹膜后腫塊可伴腎積水、輸尿管積水尾側(cè)超出骶骨岬，且不呈分葉狀-良性敏感性低，25%原發(fā)性膽管硬化膽管擴(kuò)張（膽總管狹窄）門脈高壓（門靜脈受壓）胰管局部或彌漫擴(kuò)張（硬化性胰腺炎）第11頁，共32頁。Sonography 60-year-old man with biopsy-proven idiopathic retroperitoneal fibrosis.A, Trans

5、verse sonogram at level of mid aorta reveals presence of paraaortic and preaortic hypoechoic softtissue mass (arrows). Right ureteral and pelvicalyceal dilatation were found to coexist.B, Correlating CT image also shows obstructive uropathy (arrowheads) resulting from ureteral involvement thatpreclu

6、ded contrast administration. Note that calcified abdominal aorta is not elevated from underlying lumbarspine and relatively smooth peripheral margins of abnormal soft tissue (arrows). 第12頁，共32頁。CT 病變定位、范圍、鄰近器官及血管有利于顯示病因腹主動脈瘤胰腺炎、腸系膜腺病腹膜后腫塊、位于脊柱旁、邊界清楚、形態(tài)不規(guī)則、呈等密度第13頁，共32頁。CT腹主動脈或髂動脈周圍輸尿管后腹膜腹主動脈分叉處中心向前十

7、二指腸胰腺脾臟縱隔骶骨頭尾側(cè)一般不會發(fā)生骨破壞，但惡性疾病可繼發(fā)一般良性病變中腹主動脈和下腔靜脈不會發(fā)生移位，但也有例外第14頁，共32頁。CT強(qiáng)化強(qiáng)化程度與纖維化進(jìn)展相關(guān)顯著強(qiáng)化-急性期；低強(qiáng)化或無強(qiáng)化-進(jìn)展期或慢性疾病Brun等發(fā)現(xiàn)約1/3經(jīng)手術(shù)病理證實(shí)的RPF患者無CT異常表現(xiàn)第15頁，共32頁。CT55-year-old man with retroperitoneal fibrosis.A and B, Axial oral and IV contrast-enhanced CT images show presence of low-attenuation mass anterio

8、rand lateral to aorta and iliac vessels, without anterior displacement of either aorta or inferior vena cava.Retroperitoneal mass obliterates fat plane between vessels and psoas muscle (arrows, A). Plaque bifurcates and follows mon iliac arteries (arrowhead, B).第16頁，共32頁。CT55-year-old man with infla

9、mmatory abdominal aortic aneurysm.A and B, Oral and IV contrast-enhanced axial (A)and coronal (B) CT images show ill-defined mass of soft-tissue attenuation surrounding atheromatous aneurysm. Bilateral nephrostomy tubes have been placed for obstructive uropathy.第17頁，共32頁。CT 在鑒別良惡性病變中的應(yīng)用比較困難惡性征象體積巨大，

10、占位效應(yīng)，主動脈及下腔靜脈移位（原因可能是血管后方的淋巴結(jié)腫大）易形成結(jié)節(jié)，呈分葉狀良性征象“tethering”血栓密度，向周圍浸潤、蔓延但是敏感性和特異性都較低第18頁，共32頁。CT 在鑒別良惡性病變中的應(yīng)用50-year-old man with biopsy-confirmed non-Hodgkins lymphoma.A and B, Contrast-enhanced CT scans show bulky soft-tissue mass (arrow, A) surrounding aorta and inferior vena cava. Note slight elev

11、ation of aorta from spine, feature suggestive of neoplasia.第19頁，共32頁。CT 在鑒別良惡性病變中的應(yīng)用64-year-old woman with abdominal pain.Contrast-enhanced abdominal CT scan reveals presence of retroperitoneal mass. Aorta is minimally elevated from underlying spine, raising concern for underlying neoplasia. CT-guid

12、ed biopsy and subsequent cystoscopy confirmed presence of metastatic transitional cell carcinoma of urinary bladder.第20頁，共32頁。CT 在鑒別良惡性病變中的應(yīng)用35-year-old man with HIV who presented with abdominal pain and fever. Sputum culture and chest radiography suggested tuberculosis. IV contrast-enhanced CT scan

13、 of abdomen shows nonlobulated retroperitoneal paraaortic mass of softtissueattenuation. Biopsy confirmed benign infective lymphadenopathy.第21頁，共32頁。CT 在鑒別良惡性病變中的應(yīng)用35-year-old woman with endometrial adenocarcinoma. Confluent low-attenuation retroperitoneal metastatic deposits (arrow) have appearance

14、 similar to that of retroperitoneal fibrosis.CT scan shows this soft tissue is centered on lower infrarenal aorta, has relatively smooth margins,and does not elevate aorta from spinefeatures that may allow differentiation of malignant from benign retroperitoneal fibrosis. Note associated left hydron

15、ephrosis (arrowhead).第22頁，共32頁。CT 在鑒別良惡性病變中的應(yīng)用47 year-oldman shows retroperitoneal mass that fails to elevate aorta from spine. However, this mass has suspicious lobulated anterior margin. Upper gastrointestinal endoscopy (not shown) revealed presence of gastric adenocarcinoma, with subsequent biops

16、y-proven retroperitoneal metastasis.第23頁，共32頁。CT 在鑒別良惡性病變中的應(yīng)用50-year-old woman with new diagnosis of pancreatic adenocarcinoma. CT scan shows paraaortic retroperitoneal soft-tissue mass but no elevation of aorta from spine, which suggests benign cause. However, this mass has lobulated anterior margi

17、n, which raises concern for metastatic disease. Subsequent biopsy confirmed malignant nature of this paraaortic soft tissue.第24頁，共32頁。MRI腹膜后組織結(jié)構(gòu)顯影、病因以及并發(fā)癥的顯示信號特征與其他纖維變性相似彌漫T1WI低信號增強(qiáng)可反映水腫程度慢性、非活動期的纖維組織T1、T2WI均低信號可用于評價患者對治療的反應(yīng)-水腫減輕，提示療效好強(qiáng)化程度減輕也提示好轉(zhuǎn)第25頁，共32頁。第26頁，共32頁。MRI50-year-old man with retroperit

18、oneal fibrosis. A and B, Inflammatory abdominal aneurysm and inflammatory retroperitoneal fibrosis are seen on fat-saturated axial T1 gradient-recalled echo image (A). MR images show near-circumferential paraaortic soft-tissue mass without elevation of aorta from underlying spine. Right hydronephrosis and right renal atrophy have resulted.Contrast-enhanced image (B) shows intense enhancement of retroperitoneal fibrosis, consistent with active inflammation.第27頁，共32頁。MRI60-year-old man with idiopathic retroperito