Imaging appearance of fibrosing diseases of the retroperitoneum: can a definitive diagnosis be made?

Purpose: To assess the frequency with which previously reported characteristic findings of retroperitoneal fibrosis (RPF) (a circumferential or almost circumferential peri-aortic mass centered at L4, which does not displace the abdominal aorta or proximal common iliac arteries) are present in patients with RPF, in patients with other fibrosing diseases, and in cancer patients referred to a subspecialty clinic with a suspected diagnosis of RPF, in order to determine whether diagnostic percutaneous biopsy can be avoided in some patients.

Methods: This HIPAA-compliant Institutional Review Board-approved retrospective study assessed clinical and CT and MR imaging abnormalities on imaging studies in 92 patients referred to a subspecialty clinic with suspected RPF over a 14-year period. Two reviewers, in consensus, determined the frequency of different CT and MRI findings in three groups of patients (Group 1: those with an eventual diagnosis of RPF, Group 2: those with a fibrosing disease associated with vascular or urologic abnormalities, and Group 3: those with cancer). Assessed imaging features included the presence of retroperitoneal masses, whether masses were single or multiple, whether such masses were circumferential or nearly circumferential, whether they displaced the aorta away from the spine (with the degree of such displacement measured), and whether there were abnormalities outside of the peri-aortic region of the retroperitoneum. The frequency with which findings previously reported as characteristic of RPF were present was determined for each of the three groups. Imaging results were correlated with the final diagnoses.

Results: Of 68 subjects eventually diagnosed with retroperitoneal fibrosis (RPF) (Group 1), 47 had peri-aortic retroperitoneal masses, 18 of which displaced the aorta anteriorly away from the spine. Of 12 subjects with fibrosing abnormalities related to vascular or urologic disease (Group 2), six had retroperitoneal masses, none of which displaced the aorta away from the spine. Of 12 subjects with malignancies (Group 3), six had peri-aortic retroperitoneal masses only two of whom had aortic displacement. Only 34 of 68 Group 1 subjects had peri-aortic masses characteristic of RPF, compared with six Group 2 subjects and one Group 3 subject. Subjects with characteristic retroperitoneal masses were significantly more likely to have benign disease than cancer (p = 0.009).

Conclusion: Many patients with RPF do not have characteristic imaging findings. Contrary to prior publications, absence of aortic displacement is not seen in all patients with RPF and is seen in some cancer patients. Nonetheless, when infiltrative peri-aortic retroperitoneal soft tissue that does not displace the aorta is encountered on CT or MRI, RPF can be diagnosed with a high degree of confidence, obviating the need for biopsy.