Retroperitoneal Fibrosis Clinical Presentation

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

History

The symptoms of retroperitoneal fibrosis (RPF) are vague and nonspecific. Duration of symptoms prior to diagnosis is approximately 6-12 months.[18, 19] Retroperitoneal fibrosis progresses through 2 clinical stages: (1) an early phase, which relates to the onset of inflammatory process, and (2) a late phase.

  • The most common symptom (92% of cases) is a dull, poorly localized, noncolicky pain in the flank, back, scrotum, or lower abdomen.[20] Other symptoms include fever, lower-extremity edema, phlebitis, and deep venous thrombosis. In children, pain may be referred to the ipsilateral hip or gluteal region, resulting in resistance to hip extension.[21]
  • Other uncommon presentations include weight loss, nausea, vomiting, anorexia, and malaise.
  • Rare manifestations of retroperitoneal fibrosis reported in the literature include Raynaud phenomenon, ureteric colic, hematuria, claudication, and urinary frequency. Retroperitoneal fibrosis can be associated with Crohn disease, ulcerative colitis, and sclerosing cholangitis.
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Physical

  • Approximately half of individuals with retroperitoneal fibrosis have hypertension, which is probably the result of many factors. An increase in renin release secondary to obstructive uropathy may lead to hypertension. In some patients, volume-dependent hypertension may result from obstructive nephropathy.
  • Occasionally, an abdominal mass, fever, or lower limb edema is present.
  • Obstruction of the ureters with a varying degree of renal insufficiency is the earliest and most common organ involvement.
  • Uncommon physical findings due to late complications include the following:
    • Peripheral edema or thrombosis
    • Ascites
    • Hydrocele
    • Jaundice
    • Small- or large-bowel obstruction
    • Spinal cord compression
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Causes

Sixty to 70% of cases of retroperitoneal fibrosis are idiopathic.[18, 22] However, a number of etiologic factors are known.

  • Etiologic factors associated with retroperitoneal fibrosis are as follows:
    • Malignancy
    • Inflammatory periaortitis (severe atherosclerosis)
    • Chronic retroperitoneal inflammation
    • Retroperitoneal trauma
    • Autoimmune disease
    • Irradiation
    • Certain drugs (eg, beta-blockers, methysergide, methyldopa)
  • In approximately 8% of patients, retroperitoneal fibrosis is associated with malignancy. This association is observed with lymphomas, sarcomas, and many carcinomas, including carcinomas of the breast, lung, stomach, colon, bladder, prostate, and cervix.[23] Metastatic tumor cells within the retroperitoneum cause an exuberant desmoplastic response. Malignant retroperitoneal fibrosis is radiologically distinct from retroperitoneal lymph node metastasis in that it typically displaces the ureter medically.
  • Retroperitoneal fibrosis has been associated with connective tissue disease both in children and adults.[24, 25]
  • Immune-mediated connective-tissue diseases in retroperitoneal fibrosis include the following:
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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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