Ormond’s Disease or Secondary Retroperitoneal Fibrosis? An Overview of Retroperitoneal Fibrosis

Abstract

Retroperitoneal fibrosis represents a rare inflammatory disease. About two thirds of all cases seem to be idiopathic (= Ormond's disease). The remaining one third is secondary and may be ascribed to infections, trauma, radiation therapy, malignant diseases, and the use of certain drugs. Up to 15 % of patients have additional fibrotic processes outside the retroperitoneum. The clinical symptoms of retroperitoneal fibrosis are non-specific. In sonography retroperitoneal fibrosis appears as a retroperitoneal hypoechoic mass which can involve the ureters and thus cause hydronephrosis. Intravenous urography and MR urography can demonstrate the typical triad of medial deviation and extrinsic compression of the ureters and hydronephrosis. CT and MRI are the modalities of choice for the diagnosis and follow-up of this disease. The lesion typically begins at the level of the fourth or fifth lumbar vertebra and appears as a plaque, encasing the aorta and the inferior vena cava and often enveloping and medially displacing the ureters. In unenhanced CT, retroperitoneal fibrosis appears as a mass that is isodense with muscle. When using MRI, the mass is hypointense in T 1-weighted images and of variable intensity in T 2-weighted images according to its stage: it may be hyperintense in early stages, while the tissue may have a low signal in late stages. After the administration of contrast media, enhancement is greatest in the early inflammatory phase and minimal in the late fibrotic phase. Dynamic gadolinium enhancement can be useful for assessing disease activity, monitoring response to treatment, and detecting relapse. To differentiate retroperitoneal masses, diffusion-weighted MRI may provide useful information.

Diffusion-weighted MRI in the follow-up of chronic periaortitis

“…5 Today, the assessment of the response to treatment is based on regressive disease extent in contrast-enhanced CT or MRI and regressive clinical symptoms. 7,8,14 According to the guidelines of the European Society of Urogenital Radiology (ESUR), gadolinium-based contrast agents should be omitted in patients with severely impaired renal function (estimated GFR, ,30).…”

Role of Diffusion-weighted MR Imaging in the Differentiation of Benign Retroperitoneal Fibrosis from Malignant Neoplasm: Preliminary Study

“…Also, malignant neoplasms in the para-aortacaval region (eg, lymphoma or malignant infiltration originating from the stomach, testis, kidney, pancreas, prostate, or endometrium) with plaque-like confluent tissue morphology (without fibrosis) may mimic RPF (7,8 …”

Anaplastic lymphoma kinase–positive anaplastic large cell lymphoma presenting as retroperitoneal fibrosis

“…This rare phenomenon, with an incidence of 0.1 cases per 100 000 [1], carries a strong correlation with other autoimmune conditions such as immunoglobin (Ig) G4-related sclerosing disease, sclerosing mesenteritis, sclerosing mediastinitis, primary sclerosing cholangitis, Reidel's thyroiditis, autoimmune pancreatitis, and orbital pseudotumor [2]. Most cases are idiopathic, although as many as one third of cases can be attributed to secondary causes including a variety of malignancies [3]. We present a case of a 29-yearold woman who was diagnosed with idiopathic RF until the time of autopsy when disseminated anaplastic large cell lymphoma (ALCL), anaplastic lymphoma kinase (ALK)-positive type, was discovered.…”