腹膜后纤维化

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
CT 在鉴别良恶性病变中的应用wk.baidu.com
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.
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
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
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).
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).
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
Image features
Conventional radiography Excretory urography sonography CT MRI Scintigraphy
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.
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）
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
后腹膜 腹主动脉或髂动脉周围 中心 腹主动脉分叉处 向前 输尿管 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
Sonography
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
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.
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
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 woman with endometrial adenocarcinoma. Confluent lowattenuation 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
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-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.
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
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