Retroperitoneal fibrosis: typical and atypical manifestations.

Abstract

Retroperitoneal fibrosis (RPF) is a well described clinical entity that is being diagnosed with increasing frequency. RPF is characterized by replacement of the normal tissue of the retroperitoneum with fibrosis and/or chronic inflammation. However, aetiology, clinical presentation and radiological appearance in many cases are protean. Up to 15% of patients have additional fibrotic processes outside the retroperitoneum. In the abdomen, RPF may spread contiguously to involve multiple structures in both the retroperitoneum and the peritoneal cavity or multiple non-contiguous sites may be involved. We retrospectively reviewed 30 patients (19 male, 11 female; age range 28-79 years) with biopsy proven RPF. Although we found RPF most commonly as an isolated fibrotic plaque in the lower lumbar region (18 patients), 12 patients (40%) presented with RPF in atypical locations (4 peripancreatic, 1 periduodenal, 7 pelvic). The RPF was non-malignant in 24 patients (21 idiopathic, 2 perianeurysmal, 1 ergot-derivative treatment) and malignant in six cases. We present a pictorial review of the varied appearances of RPF, concentrating on atypical sites. The radiological differential diagnosis and its appearance with various imaging modalities are discussed. Current concepts with respect to management, prognosis and treatment are summarized.

The potential role of modern US in the follow-up of patients with retroperitoneal fibrosis

“…The best RPF visualization was achieved at the level of the aortic bifurcation, which is appropriate for typical RPF location (3,5). We therefore conclude that the described US protocol can be used to visualize RPF in the typical lumbar location of patients known to have the disease.…”

Retroperitoneal fibrosis – an unsolved mystery

“…sign of RPF) with tapering of the ureter (Figure 1) at L4-L5 vertebral level (18). The sensitivity and specificity of this triad is very low since the classical sign is present in 20% of unaffected normal individuals.…”

Retroperitoneal Fibrosis Secondary to Different Etiologies (Hemilaminectomy and Hypothyroidism): Reports of Two Cases

“…[19] By CT scan, of which reported sensitivity, specificity and accuracy for the diagnosis are 83.3%, 99.7%, 93.7%, respectively, [45] RF appears as a homogeneous plaque, isodense with muscle-circulating aorta and vena cava, around the anterior and lateral sides of the abdominal aorta and the iliac arteries, without causing their displacement, as well as lymphadenopathy and tumor. [44,46] In most retroperitoneal fibrosis secondary to malignant diseases, the mass tends to displace the aorta anteriorly and the ureters laterally. In the aneurysmal forms of the disease, CT helps to evaluate the extent of aneurysmal dilatation and its potential complications; aorto-enteric fistula, hydronephrosis, and infected iliac artery aneurysms.…”