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Background
Idiopathic retroperitoneal fibrosis (RPF) is characterized by the development of a fibrotic mass surrounding the abdominal aorta and its branches. The cause is unknown.
OUTLINE
Disease Associations Laboratory/Radiologic/Other Diagnostic Testing Gross Appearance and Clinical Variants Differential Diagnosis Prognosis Treatment Commonly Used Terms Internet Links
DISEASE ASSOCIATIONS CHARACTERIZATION AMYLOIDOSIS
Amyloid A gastrointestinal amyloidosis associated with idiopathic retroperitoneal fibrosis. Report of a rare autopsy case and review of the literature.Hosaka N, Ito M, Taki Y, Iwai H, Toki J, Ikehara S.
Department of Clinical Pathology, Toyooka Hospital, Toyooka City, Hyogo, Japan.
Arch Pathol Lab Med 2003 Jun;127(6):735-8 Abstract quote We report a rare autopsy case of secondary gastrointestinal amyloid A (AA) amyloidosis associated with idiopathic retroperitoneal fibrosis (IRF) in a 67-year-old woman.
Masses were identified around the aorta and inferior vena cava in her abdomen. Histologically, plasma cell infiltration was observed within fibrotic areas. Because no specific cause for the inflammatory mass was apparent, we diagnosed it as IRF. Steroid therapy, which usually reduces IRF masses, proved ineffective, and malabsorption syndrome developed 4 years later. On autopsy, amyloid protein was present systemically in the vascular walls of several organs, and deposition was highest in the gastrointestinal mucosa. Amyloid protein was identified as AA type, strongly suggesting that the amyloidosis was secondarily induced by IRF.
To our knowledge, only 2 other cases of IRF-associated amyloidosis have been reported. Two of the 3 patients of these cases were women who showed resistance to steroid therapy.
LABORATORY/RADIOLOGIC/
OTHER TESTSCHARACTERIZATION RADIOLOGIC
Computed tomography in the diagnosis of retroperitoneal fibrosis.Dalla-Palma L, Rocca-Rossetti S, Pozzi-Mucelli RS, Rizzatto G.
Urol Radiol 1981;3(2):77-83 Abstract quote We report the findings on computed tomography (CT) in idiopathic and secondary retroperitoneal fibrosis in 7 patients. In all cases CT showed the fibrotic mass, accurately defining its extent and the involvement of adjacent anatomic structures.
The densitometric and anatomopathologic aspects were correlated; contrast uptake was less in areas thought to be dense in collagen than it was in areas with more vascular regions of inflammatory change. CT assessed the extent and nature of the fibrotic plaque.
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL
Retroperitoneal fibrosis: report of 12 cases and a review of the literature.van Bommel EF, van Spengler J, van der Hoven B, Kramer P.
Department of Internal Medicine, St. Clara Hospital, Rotterdam, The Netherlands.
Neth J Med 1991 Dec;39(5-6):338-45 Related Articles, Links
Retroperitoneal fibrosis: report of 12 cases and a review of the literature.van Bommel EF, van Spengler J, van der Hoven B, Kramer P.
Department of Internal Medicine, St. Clara Hospital, Rotterdam, The Netherlands.
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 patientsVARIANTS BILATERAL HYDRONEPHROSIS Idiopathic retroperitoneal fibrosis with bilateral lower ureteric obstruction--a case report with literature review.
Koh JS, Wong MY, Li MK, Foo KT.
Department of Urology, Singapore General Hospital, Singapore.
Singapore Med J 1998 Sep;39(9):416-7 Abstract quote Whilst prostatic enlargement remains the commonest cause of bilateral hydronephrosis in males greater than 50 years of age, other rarer conditions such as retroperitoneal fibrosis should be borne in mind.
We report a case of bilateral hydronephrosis which was eventually diagnosed as idiopathic retroperitoneal fibrosis and treated with open surgical ureterolysis with omental wrapping.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES RECTAL CANCER
Retroperitoneal fibrosis mimicking a rectal tumor: report of a case.Haciyanli M, Erkan N, Elverdi B, Avci G, Fuzun M, Dicle O.
Department of General Surgery, Dokuz Eylul University Medical Faculty, Izmir, Turkey.
Dis Colon Rectum 1998 May;41(5):664-6 Abstract quote PURPOSE: The study contained herein was undertaken to report an original case of retroperitoneal fibrosis that resembled a rectal tumor both symptomatically and radiologically.
METHOD: Reported is a case of retrorectal fibrosis with a brief literature review of the topic.
RESULT: Although many forms of retroperitoneal fibrosis have been reported, extension below the pelvic rim is very unusual. Compression of the rectum and right ureter, with constipation as a chief complaint, made this case presentation unusual. Although computerized tomographic findings and needle biopsies supported the diagnosis of retroperitoneal fibrosis, an exploratory laparotomy was necessary to rule out a malignancy and to release the ureter.
CONCLUSION: A fibrotic mass involving the retrorectal region may mimic a rectal tumor. To reach a final diagnosis, an exploratory laparotomy may be necessary, despite sophisticated evaluation techniques, because it is difficult to differentiate whether the mass is malignant.
PROGNOSIS CHARACTERIZATION GENERAL
Idiopathic retroperitoneal fibrosis: prompt diagnosis preserves organ function.Monev S.
Department of Medicine, University of Mississippi Medical Center, Jackson, USA.
Cleve Clin J Med 2002 Feb;69(2):160-6 Abstract quote Prompt diagnosis of idiopathic retroperitoneal fibrosis improves chances of preserving renal function, preventing involvement of other organs, and relieving symptoms.
Computed tomography or magnetic resonance imaging helps to exclude secondary causes, but open biopsy remains the gold standard for diagnosis.
Management typically includes surgery (ureterolysis, stent placement), and drug treatment (corticosteroids), or a combination of both. However, no surgical or medical therapy has been tested in a randomized, controlled trial.REMISSION
Retroperitoneal fibrosis: a case report of spontaneous resolution.Pierre S, Cordy PE, Razvi H.
Division of Urology, St Joseph's Health Care, London, Ontario, Canada.
Clin Nephrol 2002 Apr;57(4):314-9 Abstract quote AIM: The purpose of this case report is to document an occurrence of spontaneous resolution of idiopathic retroperitoneal fibrosis and to review the investigation and management of this unusual condition.
MATERIALS AND METHODS: A detailed case summary of a patient with retroperitoneal fibrosis is presented. Current citations in Index medicus from the English-speaking literature of relevance to the topic were reviewed.
CONCLUSIONS: In this patient who refused open surgical intervention, bilateral stent placement allowed stabilization of renal function. CT-guided biopsy did not reveal malignancy. Serial CT imaging demonstrated gradual disappearance of the retroperitoneal mass. From the literature review, spontaneous resolution of this condition appears to be a rare phenomenon. Although often utilized, CT-guided biopsy may fail to exclude the presence of malignancy. Open surgical biopsy of the retroperitoneal mass and ureterolysis remain the standard of care for operative candidates. Establishing renal drainage and considering a trial of steroids or surveillance may be an option in carefully selected individuals.
TREATMENT CHARACTERIZATION GENERAL CORTICOSTEROID
Steroid therapy for idiopathic retroperitoneal fibrosis: dose and duration.Kardar AH, Kattan S, Lindstedt E, Hanash K.
Department of Urology, King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia.
J Urol 2002 Aug;168(2):550-5 Abstract quote PURPOSE: Idiopathic retroperitoneal fibrosis is an uncommon disease of unknown etiology that may involve the ureters and other retroperitoneal structures. Surgical ureterolysis as well as medical treatment with steroids have been used to treat these patients. However, there is no agreement as to the dose and duration of steroid. We adopted a regimen of long-term steroid use in this prospective study.
MATERIALS AND METHODS: We treated 12 patients with idiopathic retroperitoneal fibrosis with a regimen of steroids during a 10-year period. Tissue diagnosis was established by biopsy of all lesions and ureteral obstruction was managed with insertion of a nephrostomy tube or a ureteral stent. The initial dose of prednisolone was 60 mg. on alternate days for 2 months and was tapered during the following 2 months to a daily dose of 5 mg. The total duration of prednisolone use was 2 years.
RESULTS: Of the 12 patients 11 who completed this treatment regimen have been followed for a duration of 26 to 132 months (median 63.1) after discontinuation of treatment. Good response in the form of relief of symptoms and regression of the mass occurred in 9 cases and there were 2 failures. In 1 case the retroperitoneal mass did not regress and surgical ureterolysis was required. In the 2nd case symptoms recurred after discontinuation of steroid and a further small dose of steroids was required. Function deteriorated in 1 of 19 functioning renal units. No steroid related serious side effects developed.
CONCLUSIONS: This regimen of steroid may be used as the primary mode of treatment for the majority of patients with idiopathic retroperitoneal fibrosis with minimal complications. Patients with idiopathic retroperitoneal fibrosis should be followed periodically for the rest of their lives.METHOTREXATE
Long-term follow-up of low-dose methotrexate therapy in one case of idiopathic retroperitoneal fibrosis.Scavalli AS, Spadaro A, Riccieri V, Ricciuti GP, Taccari E, Marini M, Zoppini A.
Institute of Rheumatology, University La Sapienza, Rome, Italy.
Clin Rheumatol 1995 Jul;14(4):481-4 Abstract quote Idiopathic retroperitoneal fibrosis (RPF) is characterized by the development of a fibrotic mass surrounding the abdominal aorta and its branches, of unknown aetiology.
Several immunological mechanisms can be operative in the pathogenesis of RPF. Based on this assumption we treated a patient affected by idiopathic RPF with low-dose methotrexate (MTX) therapy.
To our knowledge this is the first example of the effectiveness and safety of a long-term low-dose MTX treatment in the post-surgical management of RPF.SURGERY
Laparoscopic treatment of retroperitoneal fibrosis: report of two cases and review of the literature.Castilho LN, Mitre AI, Iizuka FH, Fugita OE, Colombo JR Jr, Arap S.
Division of Urology, Hospital das Clinicas, Faculty of Medicine, University of Sao Paulo, Brazil.
Rev Hosp Clin Fac Med Sao Paulo 2000 Mar-Apr;55(2):69-76 Abstract quote OBJECTIVES: We present the results of treatment by laparoscopy of two patients with retroperitoneal fibrosis and review the literature since 1992, when the first case of this disease that was treated using laparoscopy was published. We also discuss the contemporary alternatives of clinical treatment with corticosteroids and tamoxifen.
CASE REPORT: Two female patients, one with idiopathic retroperitoneal fibrosis, and other with retroperitoneal fibrosis associated with Riedel's thyroiditis, were treated using laparoscopic surgery. Both cases had bilateral pelvic ureteral obstruction and were treated using the same technique: transperitoneal laparoscopy, medial mobilization of both colons, liberation of both ureters from the fibrosis, and intraperitonealisation of the ureters. Double-J catheters were inserted before the operations and removed 3 weeks after the procedures. The first patient underwent intraperitonealisation of both ureters in a single procedure. The other had 2 different surgical procedures because of technical difficulties during the first operation. Both patients were followed for more than 1 year and recovered completely from the renal insufficiency. One of them still has occasional vague lumbar pain. There were no abnormalities in the intravenous pyelography in either case.
CONCLUSIONS: Surgical correction of retroperitoneal fibrosis, when indicated, should be attempted using laparoscopy. If possible, bilateral ureterolysis and intraperitonealisation of both ureters should be performed in the same operation.Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Eight Edition. Mosby 1996.
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.
Robbins Pathologic Basis of Disease. Sixth Edition. WB Saunders 1999.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
Basic Principles of Disease
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Commonly Used Terms
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Last Updated 6/2/2003
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