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Retroperitoneal Fibrosis Clinical Presentation

  • Author: Chandra Shekhar Biyani, MS, MBBS, DUrol, FRCS(Urol), FEBU; Chief Editor: Bradley Fields Schwartz, DO, FACS  more...
 
Updated: Jun 21, 2016
 

History and Physical Examination

The symptoms and signs associated with retroperitoneal fibrosis are nonspecific. Pain, often accompanied by significant weight loss, constipation, and constitutional symptoms, is a common presenting symptom.[26] Pain is present in 92% of cases; it is typically in the flank, back, scrotum, or lower abdomen and is dull, poorly localized, and noncolicky.[1] in children, pain may be referred to the ipsilateral hip or gluteal region, resulting in resistance to hip extension.[2]

Other manifestations may include the following:

  • Fever
  • Lower-extremity edema
  • Phlebitis
  • Deep venous thrombosis
  • Nausea, vomiting, anorexia, and malaise are uncommon
  • Raynaud phenomenon, ureteric colic, hematuria, claudication, and urinary frequency occur rarely

Retroperitoneal fibrosis can also be associated with Crohn disease, ulcerative colitis, and sclerosing cholangitis.

 
 
Contributor Information and Disclosures
Author

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

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

Disclosure: Nothing to disclose.

Coauthor(s)

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: Endourological Society, British Association of Urological Surgeons, European Association of Urology

Disclosure: Nothing to disclose.

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

Joby Taylor, FRCS, MBChB 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, United Kingdom Continence Society

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Astellas.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

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, Society of Laparoendoscopic Surgeons, Society of University Urologists, Association of Military Osteopathic Physicians and Surgeons, American Urological Association, Endourological Society

Disclosure: Nothing to disclose.

Additional Contributors

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, Association of Women Surgeons, American Society of Clinical Oncology, Society of Urology Chairpersons and Program Directors, Society of Women in Urology, Society of Government Service Urologists, American College of Surgeons, American Institute of Ultrasound in Medicine, American Urological Association, New York Academy of Sciences, 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[40]
 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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