Retroperitoneal Fibrosis (Ormond's Disease): Clinical Pathologic Study of Eight Cases

Abstract

Retroperitoneal fibrosis (RF) was first described by Albarran, a French urologist, in 1905.1 Ormond in 1948 first reported this entity in the English literature.2,3 RF is a relatively unusual condition characterized by a widespread fibrosis, usually occurring in the retroperitoneum.1-3 Also known as Ormond’s disease, sclerosing fibrosis, or sclerosing retroperitonitis, RF is composed of proliferating fibrous tissue dissecting through adipose tissue and ensheathing the abdominal aorta and the branches of the common iliac vessels. This fibrotic tissue usually involves the ureters causing their compression with secondary hydronephrosis, pyelonephritis, uremia, abdominal and back pain.1-6 Initially believed to represent an idiopathic, nonmalignant, inflammatory process of unknown etiology,7-11 it later was proven that about one third of all cases are related to specific predisposing factors (secondary RF) including previous surgery, radiation therapy, pelvic tumors, or the use of certain drugs.12,13 The etiology, clinical presentation, and radiologic features of this condition are variable, and RF is often misdiagnosed at presentation, mostly as a malignancy.