Retroperitoneal fibrosis: report of 12 cases and a review of the literature

Abstract

Twelve patients with retroperitoneal fibrosis (RPF) over a 10-yr period (1980-1990) are reviewed. The clinical manifestations, radiographic findings as well as treatment and follow-up are discussed and a review of the literature is presented. Nowadays the diagnosis can be established with near-certainty by means of computed tomographic scan (CT). In case of diagnostic problems, CT- or ultrasound guided percutaneous needle biopsy can aid the diagnosis. There is now considerable evidence that RPF can be treated effectively with corticosteroids and should be considered the treatment of choice, surgery being confined to medical failures or those who do not tolerate steroids. Early and frequent CT follow-up may demonstrate the beneficial response to steroids, thereby obviating the need for exploratory or therapeutic surgery. In steroid-resistant cases, when there is no doubt about the initial diagnosis, azathioprine or cyclophosphamide may be used. Long-term follow-up is recommended in all patients.