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Retroperitoneal Fibrosis: A Review of Clinical Features and Imaging Findings

¹ Department of Radiology, University College Hospital, Newcastle Rd., Galway, Ireland.
² Department of Radiology, Massachusetts General Hospital, Boston, MA.

View larger version (101K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A —60-year-old man with biopsy-proven idiopathic retroperitoneal fibrosis. Transverse sonogram at level of mid aorta reveals presence of paraaortic and preaortic hypoechoic soft-tissue mass (arrows). Right ureteral and pelvicalyceal dilatation were found to coexist.

View larger version (141K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B —60-year-old man with biopsy-proven idiopathic retroperitoneal fibrosis. Correlating CT image also shows obstructive uropathy (arrowheads) resulting from ureteral involvement that precluded contrast administration. Note that calcified abdominal aorta is not elevated from underlying lumbar spine and relatively smooth peripheral margins of abnormal soft tissue (arrows).

View larger version (138K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2A —55-year-old man with retroperitoneal fibrosis. Axial oral and IV contrast-enhanced CT images show presence of low-attenuation mass anterior and 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).

View larger version (137K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2B —55-year-old man with retroperitoneal fibrosis. Axial oral and IV contrast-enhanced CT images show presence of low-attenuation mass anterior and 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).

View larger version (108K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3A —55-year-old man with inflammatory abdominal aortic aneurysm. 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.

View larger version (175K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3B —55-year-old man with inflammatory abdominal aortic aneurysm. 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.

View larger version (114K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4A —50-year-old man with biopsy-confirmed non-Hodgkin's lymphoma. 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.

View larger version (120K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4B —50-year-old man with biopsy-confirmed non-Hodgkin's lymphoma. 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.

View larger version (121K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5 —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.

View larger version (113K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6 —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 soft-tissue attenuation. Biopsy confirmed benign infective lymphadenopathy.

View larger version (53K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7 —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).

View larger version (125K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8 —Contrast-enhanced CT scan in 47 year-old-man 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.

View larger version (123K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9 —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.

View larger version (119K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 10A —59-year-old man with biopsy-confirmed metastatic retroperitoneal fibrosis of unknown primary cause. Coronal (A and B) and axial (C and D) T1-weighted and steady-state free precession T2-weighted MR images show low-signal-intensity paraaortic mass (arrow,A–C) with slightly nodular outline, raising suspicion for presence of malignancy. Note bilateral ureteral dilatation (arrows, D).

View larger version (126K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 10B —59-year-old man with biopsy-confirmed metastatic retroperitoneal fibrosis of unknown primary cause. Coronal (A and B) and axial (C and D) T1-weighted and steady-state free precession T2-weighted MR images show low-signal-intensity paraaortic mass (arrow, A–C) with slightly nodular outline, raising suspicion for presence of malignancy. Note bilateral ureteral dilatation (arrows, D).

View larger version (95K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 10C —59-year-old man with biopsy-confirmed metastatic retroperitoneal fibrosis of unknown primary cause. Coronal (A and B) and axial (C and D) T1-weighted and steady-state free precession T2-weighted MR images show low-signal-intensity paraaortic mass (arrow, A–C) with slightly nodular outline, raising suspicion for presence of malignancy. Note bilateral ureteral dilatation (arrows, D).

View larger version (101K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 10D —59-year-old man with biopsy-confirmed metastatic retroperitoneal fibrosis of unknown primary cause. Coronal (A and B) and axial (C and D) T1-weighted and steady-state free precession T2-weighted MR images show low-signal-intensity paraaortic mass (arrow, A–C) with slightly nodular outline, raising suspicion for presence of malignancy. Note bilateral ureteral dilatation (arrows, D).

View larger version (87K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 11A —50-year-old man with retroperitoneal fibrosis. 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.

View larger version (38K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 11B —50-year-old man with retroperitoneal fibrosis. 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.

View larger version (88K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 12A —60-year-old man with idiopathic retroperitoneal fibrosis. Arrows indicate retroperitoneal soft-tissue mass. Fat-saturated T2-weighted image shows no significant retroperitoneal edema.

View larger version (95K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 12B —60-year-old man with idiopathic retroperitoneal fibrosis. Arrows indicate retroperitoneal soft-tissue mass. Axial unenhanced T1-weighted gradient-recalled echo (B) and contrast-enhanced fat-saturated T1-weighted gradient-recalled echo (C) acquisitions show little soft-tissue enhancement after contrast administration, which also indicates absence of associated inflammation.

View larger version (97K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 12C —60-year-old man with idiopathic retroperitoneal fibrosis. Arrows indicate retroperitoneal soft-tissue mass. Axial unenhanced T1-weighted gradient-recalled echo (B) and contrast-enhanced fat-saturated T1-weighted gradient-recalled echo (C) acquisitions show little soft-tissue enhancement after contrast administration, which also indicates absence of associated inflammation.

View larger version (118K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 13A —52-year-old man with biopsy-proven idiopathic retroperitoneal fibrosis. 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).

View larger version (85K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 13B —52-year-old man with biopsy-proven idiopathic retroperitoneal fibrosis. 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).

View larger version (118K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 13C —52-year-old man with biopsy-proven idiopathic retroperitoneal fibrosis. 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.

View larger version (81K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 13D —52-year-old man with biopsy-proven idiopathic retroperitoneal fibrosis. 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.

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