- Author: Chandra Shekhar Biyani, MS, MBBS, DUrol, FRCS(Urol), FEBU; Chief Editor: Bradley Fields Schwartz, DO, FACS more...
Retroperitoneal fibrosis (RPF) is characterized by the development of extensive fibrosis throughout the retroperitoneum, typically centered over the anterior surface of the fourth and fifth lumbar vertebrae and resulting in entrapment and obstruction of retroperitoneal structures, notably the ureters. See the image below.
Signs and symptoms
The symptoms and signs associated with RPF are nonspecific, including the following:
Deep venous thrombosis
Weight loss, nausea, vomiting, anorexia, and malaise: Uncommon presentations
Raynaud phenomenon, ureteric colic, hematuria, claudication, and urinary frequency: Rare manifestations
RPF can also be associated with Crohn disease, ulcerative colitis, and sclerosing cholangitis.
See Clinical Presentation for more detail.
The diagnosis of RPF requires a high degree of suspicion, and the evaluation of patients with presumed idiopathic RPF includes ruling out secondary RPF due to malignancy, infection, retroperitoneal injury, or drugs.
Examination in patients with retroperitoneal fibrosis includes the following findings:
Hypertension: Present in approximately 50% of affected individuals with RPF
Presence of an abdominal mass (occasionally)
Obstruction of the ureters, with varying degree of renal insufficiency: Earliest and most common organ involvement
Uncommon physical findings due to late complications include the following:
Peripheral edema or thrombosis
Small- or large-bowel obstruction
Spinal cord compression
Laboratory studies and potential results for patients with suspected RPF include the following:
Erythrocyte sedimentation rate level: Elevated
C-reactive protein level: Elevated
Urea and creatinine levels: Elevated
Complete blood count: Normocytic normochromic anemia
Alkaline phosphatase levels (also reported as a marker  )
Antinuclear antibody levels (present in 60% of cases) 
Urinalysis: Usually normal; rarely, microscopic hematuria or pyuria is seen
Plasma electrophoresis: Polyclonal hypergammaglobulinemia
The following imaging studies may be used to evaluate RPF:
Plain chest and abdominal radiography
Abdominal CT scanning: Most frequently used imaging method for diagnosis and follow-up of RPF
Nuclear imaging (eg, 67 Ga, 18 FDG): Very limited role
Positron emission tomography
Biopsy and histologic/immunohistochemical examination of the affected tissue can aid in the diagnosis and determination of the extent of the disease, which in turn affect treatment management strategies. Open biopsy can ensure a definite histologic diagnosis, but it is associated with significant morbidity. Laparoscopic biopsy is safe, minimally invasive, cost-effective, and useful in making therapeutic decisions for retroperitoneal masses.
See Workup for more detail.
There is currently no consensus on the appropriate management of patients with RPF, because no controlled therapeutic trials have been performed, and conservative therapy has occasionally yielded successful outcomes.[5, 6]
Optimal care in patients with RPF requires an integrated approach of surgical and nonsurgical therapies, and it is dependent on the stage of the disease at diagnosis. The aims of management include the following:
Preserve renal function
Prevent other organ involvement
Surgical ureterolysis has been the preferred primary mode of treatment for RPF, because it allows biopsy specimens to be obtained while ureteral obstruction is relieved. Thus, primary management of RPF consists of open biopsy, ureterolysis, and lateral/intraperitoneal transposition or omental wrapping of the involved ureter.
Other surgical interventions that may be involved in RPF include the following:
Temporizing maneuvers in the form of percutaneous nephrostomy or ureteral stenting: Recommended in the presence of obstructive uropathy
Laparoscopic surgery: Includes complex ablative and reconstructive procedures
Endourologic treatment via percutaneous balloon dilatation or endoscopic incision, dilatation, and permanent wall stent 
Long-term ureteral stenting: Reasonable approach in high-risk and elderly patients to bypass ureteral obstruction
Short-term ureteral stenting: Adjunct to open surgical procedures
Relatively newer innovative surgical techniques: Ureterolysis and wrapping with Gore-Tex, excision of the ureter and reanastomosis, posterior preperitoneal flap, and renal autotransplantation 
Medications used in the management of RPF include the following:
Glucocorticoids (eg, prednisolone, prednisone)
Immunosuppressive agents (eg, mycophenolate, azathioprine)
Estrogen receptor antagonists (eg, tamoxifen)
Empirical therapy includes corticosteroids, tamoxifen, and azathioprine. The possibility of autoallergic etiology prompted the use of corticosteroids and cytotoxic drugs in managing RPF. Glucocorticoids and azathioprine are most useful in patients with signs of inflammation.
Retroperitoneal fibrosis (RPF) is characterized by the development of extensive fibrosis throughout the retroperitoneum, typically centered over the anterior surface of the fourth and fifth lumbar vertebrae. This fibrosis leads to entrapment and obstruction of retroperitoneal structures, notably the ureters. In most cases, the etiology is unknown. However, its occasional association with autoimmune diseases and its response to corticosteroids and immunosuppressive therapy suggest it is probably immunologically mediated.[9, 10] Approximately 8% of cases are associated with metastatic malignancy.
The symptoms and signs associated with retroperitoneal fibrosis are nonspecific, and diagnosis requires a high degree of suspicion. Although a definitive diagnosis can only be made based on biopsy findings, intravenous urography may provide further support for the diagnosis of retroperitoneal fibrosis, particularly if the classic features are present. CT scanning or MRI is essential for evaluating the extent of the disease process.
The aims in management of retroperitoneal fibrosis include preserving renal function, reducing the morbidity, and suppressing inflammatory processes. Although most cases are idiopathic, precipitating causes should be excluded, particularly malignancy. Surgical ureterolysis has been the preferred primary mode of treatment because it allows biopsy specimens to be obtained while ureteral obstruction is relieved. Recently, the knowledge of a possible autoallergic etiology has prompted the use of corticosteroids and cytotoxic drugs.
Anatomy of the retroperitoneum
The retroperitoneal space is bordered anteriorly by the posterior parietal peritoneum, posteriorly by the transversalis fascia, and superiorly by the diaphragm. Inferiorly, it extends to the level of pelvic brim. The anterior and posterior layers of renal fascia (Gerota fascia) subdivide the retroperitoneal space on either side of the spine into 3 compartments. The posterior space contains the pararenal fat. The intermediate space contains the kidney, the adrenal gland, and the perirenal fat. The anterior space is more extensive.
The anterior pararenal space is bordered anteriorly by the posterior parietal peritoneum, posteriorly by the anterior layer of renal fascia, and laterally by the lateral conal fascia. The anterior pararenal space contains the extraperitoneal portions of the ascending and descending colon, the duodenum, and the pancreas. The anterior pararenal space is continuous across the midline; however, collections of fluid tend to remain ipsilateral to the site of origin. Medially, the anterior layer of renal fascia blends with the connective tissue around the aorta and the inferior vena cava. The posterior layer fuses with the psoas fascia. Laterally, both layers merge to form lateral conal fascia.
Although the exact pathogenesis of retroperitoneal fibrosis has not been definitively described, good evidence supports the suggestion that it develops as an immunologic response to antigens within atherosclerotic plaques. Autopsy and CT studies have shown that fibrosis often begins around a severely atherosclerotic aorta. Thinning or breaching of the media may allow insoluble lipids, such as ceroid, an oxidized low-density lipoprotein, to leak into periaortic tissue, stimulating an immunologic reaction. This theory is supported by the presence of circulating anticeroid antibodies and the finding of ceroid-containing macrophages in nearby lymph nodes.
For this reason, Mitchinson suggested in a 1984 report that the condition should be termed chronic periaortitis. The frequent association of retroperitoneal fibrosis with aortic aneurysm and the regression of fibrosis reported following aneurysm repair further supports this theory. However, the occurrence of retroperitoneal fibrosis in children and those without an aneurysm suggests that other factors must be involved.
In some cases, an immune reaction to an external agent may initiate fibrosis. Drugs such as beta-blockers, methysergide, and methyldopa have been implicated, possibly by acting as haptens, leading to a hypersensitivity or autoimmune reaction. The fibrous reaction associated with carcinoid tumor is believed to be the result of circulating serotonin or its metabolites. Methysergide is a strong serotonin antagonist, and the rebound release of serotonin following prolonged intake may be an alternate mechanism in this case.
The association of retroperitoneal fibrosis with other connective-tissue diseases and reported familial occurrences suggest that genetic factors may also play a role. The human leukocyte antigen (HLA)–B27 cell marker has been demonstrated in several patients with retroperitoneal fibrosis.
Investigators have coined the term hyper–immunoglobulin G4 (hyper-IgG4) syndrome owing to raised levels of IgG4 and abundant IgG4-rich plasma cells in the inflammatory infiltrate. Pathogenetic features of idiopathic retroperitoneal fibrosis include the following:
HLA-B27 or HLA-DRB1 03 haplotype
High serum IgG4 concentration
Raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level
Positive serum autoantibodies
Marked infiltration of IgG4-positive plasma cells
A 2004 case-control study increased that smoking and exposure to asbestos also increase the risk of retroperitoneal fibrosis.
Ureteral obstruction in retroperitoneal fibrosis often appears minimal despite severe renal failure. This suggests that the obstruction relates to impairment of normal ureteric peristalsis by fibrotic tissue rather than to mechanical obstruction.
Retroperitoneal fibrosis is a relatively uncommon disease. The estimated annual incidence varies from 1 per 200,000-500,000 population.[19, 20]
Because of the nonspecific nature of the symptoms, the diagnosis of retroperitoneal fibrosis is often delayed. This may lead to progressive loss of renal function.
Envelopment of the inferior vena cava and lymphatics may result in compression or thrombosis and leads to lower-limb edema. In addition, involvement of gonadal vessels may cause scrotal edema. Occasionally, the duodenum, biliary tract, pancreas, large bowel, and mesentery are involved.[21, 22]
Malignant retroperitoneal fibrosis is associated with poor prognosis, and most patients have an average survival of approximately 3-6 months. Idiopathic retroperitoneal fibrosis carries a good prognosis, with little effect on long-term morbidity or mortality.
Race-, Sex-, and Age-related Demographics
Retroperitoneal fibrosis has no reported racial predilection. The condition occurs twice as commonly in males as in females. The peak incidence of retroperitoneal fibrosis is in adults aged 40-60 years.[11, 24] Childhood presentation is extremely rare. To date, approximately 33 cases in children younger than 18 years have been reported.
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|Retroperitoneal Fibrosis||Retroperitoneal Lymphoma||Sclerosing Mesenteritis||Desmoid-Type Fibromatosis||Inflammatory Myofibroblastic Tumor||Well-Differentiated Liposarcoma Sclerosing Variant|
|Reactive perivascular lymphoid aggregates||100%||Absent||Variable||Rare||Variable||Present in the inflammatory type|
|Necrosis||Absent||Variable||Fat necrosis||Rare||Focal||Fat necrosis|
|Β-catenin||Negative||Unknown||Negative||Positive in 90% of cases||Negative||Variable positivity|
|ALK-1||Negative||Usually negative||Negative||Negative||Positive in 50% of cases||Negative|
|CD-117||Negative in spindle cell component||Rare||Variable||Negative||Rare||Negative|
|S100||Negative||Negative||Negative||Rare||Negative||Usually positive in the adipocytic component|