Retroperitoneal Fibrosis Workup

  • Author: Chandra Shekhar Biyani, MBBS, MS, DUrol, FRCS(Urol), FEBU; Chief Editor: Bradley Fields Schwartz, DO, FACS   more...
 
Updated: Jan 19, 2012
 

Laboratory Studies

  • Blood
    • An elevated ESR
    • Raised urea and creatinine levels (50%-75%)
    • Normocytic normochromic anemia
    • Raised C-reactive protein level
    • Polyclonal hypergammaglobulinemia
    • Alkaline phosphatase (has also been reported as a marker[26] )
    • Antinuclear antibodies (ANA; present in 60% of cases)[27]
  • Urinalysis
    • Results are usually normal.
    • Rarely, microscopic hematuria or pyuria is observed.
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Imaging Studies

  • Plain radiography
    • Nonspecific findings are usually due to the late complications.
    • Plain abdominal radiography may show obliteration of the psoas shadow and an enlarged renal outline due to hydronephrosis. Features of ankylosing spondylitis or metastasis may also be visible.
    • Chest radiography may demonstrate pulmonary edema or fibrosis. Mediastinal widening may result from a soft-tissue mass associated with mediastinal fibrosis.
  • Intravenous urography
    • The classic triad includes delay of contrast material with unilateral (20%) or bilateral (68%) hydronephrosis, medial deviation of the middle third of the ureters, as depicted in the image below, and tapering of the ureter at the level of L4/L5 vertebrae.[20, 28] Up to 18%-20% of control subjects may show this triad.[29] Intravenous urogram shows medial deviation of the Intravenous urogram shows medial deviation of the middle part of both ureters.
    • Medial deviation of the ureter may result from retroperitoneal neoplasm, aneurysm, and bladder diverticulum.
  • Retrograde pyelography
    • Retrograde pyelography may show features similar to those described above. In addition, it may demonstrate poor distensibility of the ureters, as depicted in the images below.Retrograde ureterogram reveals smooth narrowing anRetrograde ureterogram reveals smooth narrowing and medial shift of the ureter. Retrograde pyelogram demonstrates hydronephrosis. Retrograde pyelogram demonstrates hydronephrosis.
    • Retrograde pyelography delineates the pelvic calyceal anatomy and is usually performed prior to insertion of the stent to decompress the kidneys.
    • Interestingly, very little resistance is encountered during ureteric catheterization despite the extensive extrinsic fibrosis.
  • Lymphangiography
    • The retroperitoneal lymphatics are delicate and fine structures; therefore, they are more easily compressed by retroperitoneal fibrosis than by the adjacent blood vessels and ureters. Thus, lymphatic obstruction should precede ureteric compression.
    • Lymphangiography may show obstruction of lymphatic flow at L3/L4 level, opacification of collateral channels, nonvisualization of lymphatics above the L4 vertebra, and delay in passage of contrast through the iliac and para-aortic lymphatics.[30]
  • Ultrasonography
    • Ultrasonography is a simple noninvasive modality used to assess response to therapy.
    • On a sonogram, retroperitoneal fibrosis appears as a retroperitoneal, extensive, well-defined, hypoechoic mass centered over the sacral promontory.
    • The degree of hydronephrosis and hydroureter may vary.
    • Doppler ultrasonography has no role in differentiating benign from malignant retroperitoneal fibrosis.[31]
  • CT scanning
    • CT scanning is the most frequently used imaging method for diagnosis and follow-up of retroperitoneal fibrosis.
    • On unenhanced CT scans, retroperitoneal fibrosis appears as a plaque that is isodense with muscle and that envelops the aorta and inferior vena cava between the renal hila and sacral promontory and usually extends laterally to incorporate the ureters. Obliteration of the fat plane between the mass and the psoas muscle may be observed, as depicted in the images below.Contrast-enhanced CT scan demonstrates a periaortiContrast-enhanced CT scan demonstrates a periaortic soft tissue attenuating mass. Noncontrast CT scan shows periaortic fibrotic reacNoncontrast CT scan shows periaortic fibrotic reaction associated with an inflammatory aortic aneurysm. Note bilateral ureteric stents.
    • Retroperitoneal hemorrhage, primary retroperitoneal sarcoma, metastatic deposits to the retroperitoneum, and retroperitoneal amyloidosis may show similar findings on CT scans. CT scan features that suggest malignant pathology include lateral displacement of the ureter, anterior displacement of the aorta, local bone destruction, and a large bulky lesion.
    • Elevation of the aorta from the spine is uncommon in benign retroperitoneal fibrosis. If present, it may be a sign of malignancy.[32]
    • After contrast injection, the plaque may show a variable degree of enhancement, depending on the stage of the disease. Enhancement is usually significant in the early active vascular stage. On the other hand, enhancement is poor in the late vascular stage.
  • Magnetic resonance imaging
    • Both benign and malignant retroperitoneal fibrosis have low-to-intermediate signal density on T1-weighted images and density on T2-weighted images that varies based on the disease stage. During the early stage, signal density is high because of high fluid content and hypercellularity. In contrast, late-stage disease has low T2 signal intensity as a result of avascular acellular fibrosis and decreased fluid content.[33] Steroid therapy may lead to similar changes because of decreased tissue edema.
    • Inhomogeneity of signal intensity on T2-weighted images may suggest malignancy; however, differentiation between benign and malignant retroperitoneal fibrosis on MRI is difficult, and biopsy is usually required to confirm the diagnosis.[15]
    • One study assessed delayed gadolinium enhancement ratios by comparing retroperitoneal fibrosis enhancement with that of psoas muscle. The dynamic enhancement ratio in acute retroperitoneal fibrosis significantly differed from the ratio in chronic retroperitoneal fibrosis. Dynamic gadolinium enhancement may be useful in differentiating newly diagnosed retroperitoneal fibrosis from treated chronic disease and may have a role in assessing disease activity, monitoring response to treatment, and detecting relapse.[34]
    • MRI has some advantages over CT scanning, including multiplanar capability, independence of renal function, and absence of radiation.
  • Nuclear medicine
    • Nuclear medicine has a very limited role.
    • In the acute phase, retroperitoneal fibrosis may take up gallium-67, possibly because of the binding of gallium-67 to lymphocytes.[35]
    • A small study of 2-deoxy-2-(F-18) fluoro-D-glucose (F-18DG) positron emission tomography (PET) scanning reported a low F-18DG uptake in retroperitoneal fibrosis and high uptake in malignant lymphoma.[36]
  • Positron emission tomography
    • PET scanning with 18F-fluorodeoxyglucose (18F-FDG) is a functional imaging modality used in oncology, but recent evidence suggests that it can be useful in the evaluation of various inflammatory diseases.[37]
    • 18F-FDG identifies areas of high glucose metabolic activity. Because inflammatory cells have an increased glucose uptake, high levels of glucose metabolism are seen in a retroperitoneal mass associated with retroperitoneal fibrosis if inflammation is present.
    • No noninvasive method can reliably assess disease activity. However, 18F-FDG is useful for evaluating posttreatment disease. In addition, it can reveal other sites of disease (thyroid, thorax) and may help to identify the most appropriate sites for retroperitoneal biopsy.
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Other Tests

  • Biopsy
    • A wide spectrum of fibroinflammatory disorders can mimic retroperitoneal fibrosis on imaging. Management strategies are diverse and depend strongly on the histological diagnosis and extent of the disease. Histology and immunohistochemistry are required to confirm the diagnosis. The amount of tissue harvested via core needle biopsy may not be sufficient for the histological diagnosis. Therefore, open biopsy can ensure a definite histological diagnosis and is traditionally performed. However, it is associated with significant morbidity. Laparoscopic biopsy is safe, minimally invasive, cost-effective, and useful in making therapeutic decisions for retroperitoneal masses.
    • Tissue for histologic diagnosis can be obtained under CT or ultrasonographic guidance. In a 1999 publication, Dash et al described fine-needle aspiration for the diagnosis of retroperitoneal fibrosis, but most clinicians prefer a Tru-Cut needle biopsy.[38]
    • In 1998, Pfammatter et al performed transcaval retroperitoneal core biopsies and suggested that the technique may have a role in patients at high operative risk, especially if the results from standard biopsies are inconclusive.[39]
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Histologic Findings

In 1948, Ormond described two histologic features in retroperitoneal fibrosis: an inflammatory early stage and a chronic stage.[40] In the early stage, an inflammatory infiltrate contains macrophages, lymphocytes, plasma cells, and occasional eosinophils; neutrophils are generally absent. The macrophages are often lipid-laden and contain areas of perivascular lymphocytic infiltrate composed of T cells and B cells. Generally, tissue is highly vascular with numerous small blood vessels throughout. In the chronic stage, the tissue becomes avascular and acellular with scattered calcification and progresses to fibrous scarring.[41] Occasionally, surrounding structures are invaded by retroperitoneal fibrosis. Invasion of the large veins may cause fibrous thickening of the intima, resulting in complete occlusion. Periaortic lymphatics may be blocked within the mass. Submucosal edema and lymphocytic infiltration may be observed in the ureter.

Corradi et al (2007) recently reported the presence of CD20 and CD3 cells, IgG4 plasma cells, and subtle vasculitic activity in idiopathic retroperitoneal fibrosis.[42]

Malignant retroperitoneal fibrosis demonstrates the presence of scattered nests of malignant cells within the inflammatory infiltrate. Hodgkin disease and sclerosing retroperitoneal lymphomas are the most challenging differential diagnoses for the pathologist to exclude. In 2002, Wu et al recommended the use of immunostains such as c-Kit, Leu-M1, Ki-1, LCA, and kappa and lambda light chain.[43]

Table. Differential Diagnoses of Retroperitoneal Fibrosis[42] (Open Table in a new window)

Retroperitoneal FibrosisRetroperitoneal LymphomaSclerosing MesenteritisDesmoid-Type FibromatosisInflammatory Myofibroblastic TumorWell-Differentiated Liposarcoma Sclerosing Variant
Ureteral displacementMedialLateral
Ureteral obstruction~80%~50%RareRareRareUnknown
Aortic displacementRareAnterior
Reactive perivascular lymphoid aggregates100%AbsentVariableRareVariablePresent in the inflammatory type
NecrosisAbsentVariableFat necrosisRareFocalFat necrosis
Vasculitis~50%AbsentAbsentAbsentAbsentAbsent
ClonalityAbsentVariableAbsentAbsentAbsentPresent
Β-cateninNegativeUnknownNegativePositive in 90% of casesNegativeVariable positivity
ALK-1NegativeUsually negativeNegativeNegativePositive in 50% of casesNegative
CD-117Negative in spindle cell componentRareVariableNegativeRareNegative
DesminNegativeNegativeVariableRareUsually positiveRare
S100NegativeNegativeNegativeRareNegativeUsually positive in the adipocytic component
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Contributor Information and Disclosures
Author

Chandra Shekhar Biyani, MBBS, MS, DUrol, FRCS(Urol), FEBU  Consulting Urologist, Department of Urology, Pinderfields General Hospital, The Mid-Yorkshire Hospitals NHS Trust, UK

Chandra Shekhar Biyani, MBBS, MS, DUrol, FRCS(Urol), FEBU is a member of the following medical societies: British Association of Urological Surgeons, British Medical Association, European Association of Urology, and International College of Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Joby Taylor, MBChB, FRCS  Consultant Urologist, Forth Valley Royal Hospital, UK

Joby Taylor, MBChB, FRCS is a member of the following medical societies: British Association of Urological Surgeons, European Association of Urology, International Continence Society, Royal College of Surgeons of Edinburgh, and United Kingdom Continence Society

Disclosure: Nothing to disclose.

Anthony J Browning, MB, ChB, FRCS(Edin)  Consultant Urological Surgeon, Mid Yorkshire NHS Trust, Pinderfields General Hospital; Associate Post Graduate Dean, Yorkshire and Humber Deanery, UK

Anthony J Browning, MB, ChB, FRCS(Edin) is a member of the following medical societies: British Association of Urological Surgeons, Endourological Society, and European Association of Urology

Disclosure: Nothing to disclose.

Specialty Editor Board

Martha K Terris, MD, FACS  Professor, Department of Surgery, Section of Urology, Director, Urology Residency Training Program, Medical College of Georgia; Professor, Department of Physician Assistants, Medical College of Georgia School of Allied Health; Chief, Section of Urology, Augusta Veterans Affairs Medical Center

Martha K Terris, MD, FACS is a member of the following medical societies: American Cancer Society, American College of Surgeons, American Institute of Ultrasound in Medicine, American Society of Clinical Oncology, American Urological Association, Association of Women Surgeons, New York Academy of Sciences, Society of Government Service Urologists, Society of University Urologists, Society of Urology Chairpersons and Program Directors, and Society of Women in Urology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Martin I Resnick, MD †  Former Lester Persky Professor and Chair, Department of Urology, Former Professor, Department of Oncology, Case Western Reserve University School of Medicine

Martin I Resnick, MD † is a member of the following medical societies: American College of Surgeons, American Federation for Medical Research, American Institute of Ultrasound in Medicine, American Medical Association, American Society for Bone and Mineral Research, American Society for Reproductive Medicine, American Society of Andrology, American Surgical Association, American Urological Association, Association for Academic Surgery, Endocrine Society, National Kidney Foundation, Ohio Urological Society, and Pan American Medical Association

Disclosure: Nothing to disclose.

J Stuart Wolf Jr, MD, FACS  The David A Bloom Professor of Urology, Director, Division of Endourology and Stone Disease, Department of Urology, University of Michigan Medical School

J Stuart Wolf Jr, MD, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Catholic Medical Association, Endourological Society, Society for Urology and Engineering, Society of Laparoendoscopic Surgeons, Society of University Urologists, and Society of Urologic Oncology

Disclosure: Nothing to disclose.

Chief Editor

Bradley Fields Schwartz, DO, FACS  Professor of Urology, Director, Center for Laparoscopy and Endourology, Department of Surgery, Southern Illinois University School of Medicine

Bradley Fields Schwartz, DO, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Association of Military Osteopathic Physicians and Surgeons, Endourological Society, Society of Laparoendoscopic Surgeons, and Society of University Urologists

Disclosure: Nothing to disclose.

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Intravenous urogram shows medial deviation of the middle part of both ureters.
Retrograde ureterogram reveals smooth narrowing and medial shift of the ureter.
Retrograde pyelogram demonstrates hydronephrosis.
Contrast-enhanced CT scan demonstrates a periaortic soft tissue attenuating mass.
Noncontrast CT scan shows periaortic fibrotic reaction associated with an inflammatory aortic aneurysm. Note bilateral ureteric stents.
Management algorithm of retroperitoneal fibrosis.
Postureterolysis intravenous urogram demonstrates lateral displacement of both ureters and a double J stent on the right side.
Retrograde pyelogram shows satisfactory positioning of a wall stent in a patient with postureterolysis obstruction.
Abdominal radiograph demonstrates a wall stent on the right side.
Table. Differential Diagnoses of Retroperitoneal Fibrosis[42]
Retroperitoneal FibrosisRetroperitoneal LymphomaSclerosing MesenteritisDesmoid-Type FibromatosisInflammatory Myofibroblastic TumorWell-Differentiated Liposarcoma Sclerosing Variant
Ureteral displacementMedialLateral
Ureteral obstruction~80%~50%RareRareRareUnknown
Aortic displacementRareAnterior
Reactive perivascular lymphoid aggregates100%AbsentVariableRareVariablePresent in the inflammatory type
NecrosisAbsentVariableFat necrosisRareFocalFat necrosis
Vasculitis~50%AbsentAbsentAbsentAbsentAbsent
ClonalityAbsentVariableAbsentAbsentAbsentPresent
Β-cateninNegativeUnknownNegativePositive in 90% of casesNegativeVariable positivity
ALK-1NegativeUsually negativeNegativeNegativePositive in 50% of casesNegative
CD-117Negative in spindle cell componentRareVariableNegativeRareNegative
DesminNegativeNegativeVariableRareUsually positiveRare
S100NegativeNegativeNegativeRareNegativeUsually positive in the adipocytic component
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