腹膜后纖維化

1、腹膜后纖維化,腹組讀片劉婧2010-6-23,Introduction,腹膜后纖維化retroperitoneal fibrosis（RPF）少見的纖維化反應（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

2、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,Introduction,惡性腹膜后纖維化占8%，預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特征是關鍵,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伴發(fā)罕有兒童及家族遺傳病例報道,Epidemiology,尿

3、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展期動脈粥樣硬化的嚴重炎癥反應假定的過敏原為粥樣斑塊產(chǎn)生的臘樣色素,,,與腹膜后纖維化相關的免疫疾病,原發(fā)性膽管硬化纖維化縱膈炎腎小球腎炎風濕性關節(jié)炎系統(tǒng)性紅斑狼瘡結(jié)節(jié)性動脈周圍炎強直性脊柱炎橋本甲狀腺炎全垂體機能減退硬化性腸系膜炎,Clinical

4、 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,惡性淋巴瘤（Hodgkin’s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)傷或手術炎癥Crohn’s病放化療后感染結(jié)核、尿路感染腎臟創(chuàng)傷,Pathology,大體蒼白、血栓樣腫塊；邊界不清；

5、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胞浸潤，如淋巴細胞、巨噬細胞及血管內(nèi)皮細胞,Image features,Conventional radiographyExcretory urographysonographyCTMRIScintigraphy,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

6、發(fā)性膽管硬化膽管擴張（膽總管狹窄）門脈高壓（門靜脈受壓）胰管局部或彌漫擴張（硬化性胰腺炎）,Sonography,60-year-old man with biopsy-proven idiopathic retroperitoneal fibrosis.A, Transverse sonogram at level of mid aorta reveals presence of paraaortic and preaorti

7、c 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 thatprecl

8、uded contrast administration. Note that calcified abdominal aorta is not elevated from underlying lumbarspine and relatively smooth peripheral margins of abnormal soft tissue (arrows).,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

9、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,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,CT,強化強化程度與纖維化進展相關顯著強化---急性期；低強化或無強化---進展期或慢性疾病Brun等發(fā)現(xiàn)約1/3經(jīng)手術病理證實的RPF患者無

10、CT異常表現(xiàn),CT,55-year-old man with retroperitoneal fibrosis.A and B, Axial oral and IV contrast-enhanced CT images show presence of low-attenuation mass anteriorand lateral to aorta and iliac vessels, without anterior displa

11、cement of either aorta or inferior vena cava.Retroperitoneal mass obliterates fat plane between vessels and psoas muscle (arrows, A). Plaque bifurcates and follows common iliac arteries (arrowhead, B).,CT,55-year-old man

12、 with inflammatory 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 nephros

13、tomy tubes have been placed for obstructive uropathy.,CT 在鑒別良惡性病變中的應用,比較困難惡性征象體積巨大，占位效應，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,CT 在鑒別良惡性病變中的應用,50-year-old man with biopsy-con

14、firmed non-Hodgkin’s lymphoma.A and B, Contrast-enhanced CT scans show bulky soft-tissue mass (arrow, A) surrounding aorta and inferior vena cava. Note slight elevation of aorta from spine, feature suggestive of neoplasi

15、a.,CT 在鑒別良惡性病變中的應用,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 neo

16、plasia. CT-guided biopsy and subsequent cystoscopy confirmed presence of metastatic transitional cell carcinoma of urinary bladder.,CT 在鑒別良惡性病變中的應用,35-year-old man with HIV who presented with abdominal pain and fever. Sp

17、utum culture and chest radiography suggested tuberculosis. IV contrast-enhanced CT scan of abdomen shows nonlobulated retroperitoneal paraaortic mass of softtissueattenuation. Biopsy confirmed benign infective lymphadeno

18、pathy.,CT 在鑒別良惡性病變中的應用,35-year-old woman with endometrial adenocarcinoma. Confluent low-attenuation retroperitoneal metastatic deposits (arrow) have appearance similar to that of retroperitoneal fibrosis.CT scan shows th

19、is soft tissue is centered on lower infrarenal aorta, has relatively smooth margins,and does not elevate aorta from spine—features that may allow differentiation of malignant from benign retroperitoneal fibrosis. Note as

20、sociated left hydronephrosis (arrowhead).,CT 在鑒別良惡性病變中的應用,47 year-oldman shows retroperitoneal mass that fails to elevate aorta from spine. However, this mass has suspicious lobulated anterior margin. Upper gastrointesti

21、nal endoscopy (not shown) revealed presence of gastric adenocarcinoma, with subsequent biopsy-proven retroperitoneal metastasis.,CT 在鑒別良惡性病變中的應用,50-year-old woman with new diagnosis of pancreatic adenocarcinoma. CT scan

22、shows paraaortic retroperitoneal soft-tissue mass but no elevation of aorta from spine, which suggests benign cause. However, this mass has lobulated anterior margin, which raises concern for metastatic disease. Subseque

23、nt biopsy confirmed malignant nature of this paraaortic soft tissue.,MRI,腹膜后組織結(jié)構顯影、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----水腫減輕，提示療效好強化程度減輕也提示好轉(zhuǎn),MRI,50-year-old man with ret

24、roperitoneal 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 paraaorti

25、c 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, consiste

26、nt with active inflammation.,MRI,60-year-old man with idiopathic retroperitoneal fibrosis. Arrows indicate retroperitoneal soft-tissue mass. A, Fat-saturated T2-weighted image shows no significant retroperitoneal edema.

27、B and C, Axial unenhanced T1-weighted radientrecalled echo (B) and contrast-enhanced fatsaturated T1-weighted gradient-recalled echo © acquisitions show little soft-tissue enhancement after contrast administration,

28、which also indicates absence of associated inflammation.,Scintigraphy,67Ga、FDG PET攝取可反映炎癥階段顯著攝取----早期、炎癥階段少量或不攝取----纖維化階段,Management option,活檢開腹活檢、腹腔鏡活檢、影像引導下活檢腫瘤細胞分散于纖維塊中----陰性不代表良性消除病因是關鍵皮質(zhì)類固醇免疫抑制劑外科干預并發(fā)癥治療,Ou