A computerized tomography (CT) scan represents the most commonly used imaging technique for diagnosis. Magnetic resonance imaging (MRI) is unable to differentiate more accurately between benign and malignant retroperitoneal fibrosis (RF) than a CT scan.

Biopsy remains the most reliable diagnostic tool for IRF. However, the histological characteristics of IRF are not yet well-defined and the protocol for biopsy is not standardized in terms of template, number of biopsies and the immunohistochemical panel needed for positive diagnosis.

The most common treatment reported is corticosteroid therapy alone or in combination with other immunosuppressants, whereas surgical treatment is reserved for severe cases. Indwelling ureteric stents represent the most common procedure for renal drainage, but their efficacy is questionable.

Open ureterolysis remains the gold standard for surgical treatment, but its purpose is only to resolve the ureteric obstruction, not to treat the retroperitoneal fibrosis. Laparoscopic and robotic approaches have been reported to be feasible, but no prospective, comparative trials have been performed due to the rarity of the disease.

Surgical technique is not standardized and the outcome of the treatment only evaluates the recovery of the renal function.

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