1、腹膜后纤维化腹膜后纤维化Introduction o 腹膜后纤维化腹膜后纤维化retroperitoneal fibrosis（RPF）o 少见的纤维化反应（胶原血管病）o 1905，由法国泌尿科医生Albarran首次介绍n腹膜后纤维化反应继发输尿管狭窄o 1948，Ormond首次提出RPF的概念o 慢性主动脉周炎、输尿管周炎、硬化性腹膜后肉芽肿慢性主动脉周炎、输尿管周炎、硬化性腹膜后肉芽肿o 因输尿管梗阻是常见的后遗症，排泄性尿路造影、逆排泄性尿路造影、逆行性肾盂造影行性肾盂造影可用于发现梗阻的部位及程度o CT、MR成为诊断该病及评价继发表现的主力o18F-FDG PET核素显像核素显
2、像2腹膜后纤维化Introduction o恶性腹膜后纤维化占8%，预后差o熟悉其潜在的临床表现潜在的临床表现以及典型或不典型的影像学特征影像学特征是关键3腹膜后纤维化Epidemiologyo 发病率为1/200，000o 好发年龄：40-60；男女比例（2-3）：1o 绝大部分病例（70%）为特发性腹膜后纤维化o 其余常与感染、恶性肿瘤以及药物介导伴发o 罕有儿童及家族遗传病例报道4腹膜后纤维化Epidemiologyo 尿路梗阻性疾病尿路梗阻性疾病 全身系统性疾病全身系统性疾病o 常与各种免疫紊乱伴发以及对免疫抑制剂有效 自身免疫病因假说自身免疫病因假说o 腹膜后纤维化是对进展期动脉粥样
3、硬化动脉粥样硬化的严重炎症反严重炎症反应应o 假定的过敏原为粥样斑块产生的腊样色素腊样色素5腹膜后纤维化与腹膜后纤维化相关的免疫疾病与腹膜后纤维化相关的免疫疾病 原发性胆管硬化原发性胆管硬化 纤维化纵膈炎纤维化纵膈炎 肾小球肾炎肾小球肾炎 风湿性关节炎风湿性关节炎 系统性红斑狼疮系统性红斑狼疮 结节性动脉周围炎结节性动脉周围炎 强直性脊柱炎强直性脊柱炎 桥本甲状腺炎桥本甲状腺炎 全垂体机能减退全垂体机能减退 硬化性肠系膜炎硬化性肠系膜炎6腹膜后纤维化Clinical presentationo早期症状早期症状n 腹部或腰部不适n 下肢水肿或不适（淋巴管闭塞）o晚期症状晚期症状n 深静脉血栓n
4、少尿、无尿n 恶心、呕吐、尿毒症表现n 高血压n 肠系膜缺血n 肠梗阻7腹膜后纤维化o 恶性n 淋巴瘤（Hodgkins淋巴瘤）n 转移（结肠、乳腺、肺、泌尿系统、甲状腺原发瘤）n 腹膜后肉瘤n 类癌o 出血n 主动脉瘤n 动脉周围血肿n 创伤或手术o 炎症n Crohns病o 放化疗后o 感染n 结核、尿路感染o 肾脏创伤8腹膜后纤维化Pathologyo大体n 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o镜下n 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9腹膜后纤维化Image featuresoConventional radiographyoExcr
5、etory urographyosonographyoCToMRIoScintigraphy 10腹膜后纤维化Sonographyo低回声或无回声、边界清楚、形态不规则腹膜后肿块o可伴肾积水、输尿管积水o 尾侧超出骶骨岬，且不呈分叶状-良性o 敏感性低，25%11腹膜后纤维化Sonography 60-year-old man with biopsy-proven idiopathic retroperitoneal fibrosis.A,Transverse sonogram at level of mid aorta reveals presence of paraaortic and p
6、reaortic 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 thatprecluded contrast administration.Note that calcified abdominal aorta is not elevate
7、d from underlying lumbarspine and relatively smooth peripheral margins of abnormal soft tissue(arrows).12腹膜后纤维化CT o病变定位、范围、邻近器官及血管o有利于显示病因o腹主动脉瘤o胰腺炎、肠系膜腺病o腹膜后肿块、位于脊柱旁、边界清楚、形腹膜后肿块、位于脊柱旁、边界清楚、形态不规则、呈等密度态不规则、呈等密度13腹膜后纤维化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不会发生骨破坏，但恶性疾病可继发一般良
8、性病变中腹主动脉和下腔静脉不会发生移位，但也有例外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14腹膜后纤维化CTo强化n 强化程度与纤维化进展相关n 显著强化-急性期；n 低强化或无强化-进展期或慢性疾病oBrun等发现约1/3经手术病理证实的RPF患者无CT异常表现15腹膜后纤维化CT55-year-old man with retroperitoneal fibrosis.A and B,Axial oral and IV contrast-enhanced CT images show presence of low-attenuation mass anteriorand
9、 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 common iliac arteries(arrowhead,B).16腹膜后纤维化CT55-year-old man with inflammatory a
10、bdominal 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.17腹膜后纤维化CT 在鉴别良恶性病变中的应用在鉴别良恶性病变中的应用o比较困难o恶性征象n 体积巨大，
11、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）n 易形成结节，呈分叶状o良性征象n“tethering”n 血栓密度，向周围浸润、蔓延o但是敏感性和特异性都较低18腹膜后纤维化CT 在鉴别良恶性病变中的应用在鉴别良恶性病变中的应用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 con
12、cern for underlying neoplasia.CT-guided biopsy and subsequent cystoscopy confirmed presence of metastatic transitional cell carcinoma of urinary bladder.20腹膜后纤维化CT 在鉴别良恶性病变中的应用在鉴别良恶性病变中的应用35-year-old man with HIV who presented with abdominal pain and fever.Sputum culture and chest radiography sugges
13、ted tuberculosis.IV contrast-enhanced CT scan of abdomen shows nonlobulated retroperitoneal paraaortic mass of softtissueattenuation.Biopsy confirmed benign infective lymphadenopathy.21腹膜后纤维化CT 在鉴别良恶性病变中的应用在鉴别良恶性病变中的应用35-year-old woman with endometrial adenocarcinoma.Confluent low-attenuation retrop
14、eritoneal metastatic deposits(arrow)have appearance 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 ret
15、roperitoneal fibrosis.Note associated left hydronephrosis(arrowhead).22腹膜后纤维化CT 在鉴别良恶性病变中的应用在鉴别良恶性病变中的应用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 pres
16、ence of gastric adenocarcinoma,with subsequent biopsy-proven retroperitoneal metastasis.23腹膜后纤维化CT 在鉴别良恶性病变中的应用在鉴别良恶性病变中的应用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 b
17、enign cause.However,this mass has lobulated anterior margin,which raises concern for metastatic disease.Subsequent biopsy confirmed malignant nature of this paraaortic soft tissue.24腹膜后纤维化MRIo 腹膜后组织结构显影、病因以及并发症的显示o 信号特征与其他纤维变性相似n 弥漫T1WI低信号n 增强可反映水肿程度n 慢性、非活动期的纤维组织T1、T2WI均低信号n 可用于评价患者对治疗的反应-水肿减轻，提示疗效
18、好n 强化程度减轻也提示好转25腹膜后纤维化26腹膜后纤维化MRI50-year-old man with retroperitoneal 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
19、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.27腹膜后纤维化MRI60-year-old man with idiopathic retroperitoneal fibrosis.Arrows indicate
20、retroperitoneal soft-tissue mass.A,Fat-saturated T2-weighted image shows no significant retroperitoneal edema.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,which also indicates absence of associated inflammation.28腹膜后纤维化Scintigraphy o67Ga、FDG PET摄取可反映炎症阶段n 显著摄取-早期、炎症阶段n 少量或不摄取-纤维化阶段29腹膜后纤维化30腹膜后纤维化Management optiono活检n 开腹活检、腹腔镜活检、影像引导下活检o肿瘤细胞分散于纤维块中-阴性不代表良性o消除病因是关键n 皮质类固醇n 免疫抑制剂n 外科干预o并发症治疗31腹膜后纤维化Outcome o良性预后好o恶性预后差：3-6个月32腹膜后纤维化
