腹膜后纤维化 腹组读片 刘婧 2010-6-23

Introduction 腹膜后纤维化retroperitoneal fibrosis（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-FDG PET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 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

Clinical presentation 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 镜下 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 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

Image features Conventional radiography Excretory urography sonography CT MRI Scintigraphy

Sonography 尾侧超出骶骨岬，且不呈分叶状----良性 敏感性低，25% 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）

Sonography — 60-year-old man with biopsy-proven idiopathic retroperitoneal fibrosis. A, Transverse 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 thatprecluded 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 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 后腹膜 腹主动脉或髂动脉周围 输尿管 中心 十二指肠 胰腺 脾脏 向前 腹主动脉分叉处 头尾侧 纵隔 骶骨 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 Brun等发现约1/3经手术病理证实的RPF患者无CT异常表现 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

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 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).

CT 55-year-old man 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 nephrostomy tubes have been placed for obstructive uropathy.

CT 在鉴别良恶性病变中的应用 比较困难 恶性征象 良性征象 但是敏感性和特异性都较低 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-old man with biopsy-confirmed 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 neoplasia.

CT 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 concern for underlying neoplasia. CT-guided biopsy and subsequent cystoscopy confirmed presence of metastatic transitional cell carcinoma of urinary bladder.

CT 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 suggested tuberculosis. IV contrast-enhanced CT scan of abdomen shows nonlobulated retroperitoneal paraaortic mass of softtissueattenuation. Biopsy confirmed benign infective lymphadenopathy.

CT 在鉴别良恶性病变中的应用 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 this 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 associated left hydronephrosis (arrowhead).

CT 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 presence of gastric adenocarcinoma, with subsequent biopsy-proven retroperitoneal metastasis.

CT 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 benign cause. However, this mass has lobulated anterior margin, which raises concern for metastatic disease. Subsequent biopsy confirmed malignant nature of this paraaortic soft tissue.

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

MRI 50-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 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.

60-year-old man with idiopathic retroperitoneal fibrosis 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. 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. MRI Fig. 12—

Scintigraphy 67Ga、FDG PET摄取可反映炎症阶段 显著摄取----早期、炎症阶段 少量或不摄取----纤维化阶段

52-year-old man with biopsy-proven idiopathic retroperitoneal fibrosis 52-year-old man with biopsy-proven idiopathic retroperitoneal fibrosis.A and B, Coronal fused PET/CT (A) and coronal PET (B) images show increased paraaortic uptake of 18F-FDG,consistent with active inflammation of retroperitoneal fibrosis (white arrows). Mild right-sided ureteral obstruction is also shown on PET image (B) (black arrow, B). C and D, Follow-up PET/CT (C) and coronal PET (D) images 2 months after immunotherapy show residual paraaortic tissue (arrows, C) but no radiotracer uptake, thus indicating favorable response to treatment.

Management option 活检 肿瘤细胞分散于纤维块中----阴性不代表良性 消除病因是关键 并发症治疗 开腹活检、腹腔镜活检、影像引导下活检 肿瘤细胞分散于纤维块中----阴性不代表良性 消除病因是关键 皮质类固醇 免疫抑制剂 外科干预 并发症治疗

Outcome 良性预后好 恶性预后差：3-6个月