Diagnostic Considerations

In the evaluation of patients with presumed idiopathic retroperitoneal fibrosis (RPF), exclude secondary RPF due to malignancy, infection, retroperitoneal injury, or drugs.

RPF can be a feature of Erdheim-Chester disease, a rare form of histiocytosis that is often characterized by symmetric osteosclerosis but may also manifest as diabetes insipidus, exophthalmos, xanthelasmas, cardiovascular involvement, bilateral adrenal enlargement, renal impairment, testis infiltration, or interstitial lung disease. Affected tissues demonstrate xanthomatous infiltration by foamy histiocytes, surrounded by fibrosis.^[37]

Workup

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Media Gallery

Intravenous urogram in a patient with retroperitoneal fibrosis shows medial deviation of the middle part of both ureters.
Retrograde ureterogram in a patient with retroperitoneal fibrosis reveals smooth narrowing and medial shift of the ureter.
Retroperitoneal fibrosis. Retrograde pyelogram demonstrates hydronephrosis.
Retroperitoneal fibrosis. Contrast-enhanced CT scan demonstrates a periaortic soft tissue attenuating mass.
Retroperitoneal fibrosis. 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 in a patient with retroperitoneal fibrosis demonstrates lateral displacement of both ureters and a double J stent on the right side.
Retroperitoneal fibrosis. Retrograde pyelogram shows satisfactory positioning of a wall stent in a patient with postureterolysis obstruction.
Retroperitoneal fibrosis. Abdominal radiograph demonstrates a wall stent on the right side.

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Table. Differential Diagnoses of Retroperitoneal Fibrosis^[51]

Table. Differential Diagnoses of Retroperitoneal Fibrosis ^[51]

	Retroperitoneal Fibrosis	Retroperitoneal Lymphoma	Sclerosing Mesenteritis	Desmoid-Type Fibromatosis	Inflammatory Myofibroblastic Tumor	Well-Differentiated Liposarcoma Sclerosing Variant
Ureteral displacement	Medial	Lateral
Ureteral obstruction	~80%	~50%	Rare	Rare	Rare	Unknown
Aortic displacement	Rare	Anterior
Reactive perivascular lymphoid aggregates	100%	Absent	Variable	Rare	Variable	Present in the inflammatory type
Necrosis	Absent	Variable	Fat necrosis	Rare	Focal	Fat necrosis
Vasculitis	~50%	Absent	Absent	Absent	Absent	Absent
Clonality	Absent	Variable	Absent	Absent	Absent	Present
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
Desmin	Negative	Negative	Variable	Rare	Usually positive	Rare
S100	Negative	Negative	Negative	Rare	Negative	Usually positive in the adipocytic component

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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)

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

Disclosure: Nothing to disclose.

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

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Endourological Society Board of Directors; President Elect North Central Section of the American Urological Association<br/>Serve(d) as a speaker or a member of a speakers bureau for: Cook Medical.

Additional Contributors

Martha K Terris, MD, FACS Professor and Chief of Urology, Witherington Distinguished Chair, Department of Surgery, Section of Urology, Director, Urology Residency Training Program, Medical College of Georgia at Augusta University; 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, Society of Women in Urology

Disclosure: Nothing to disclose.

Retroperitoneal Fibrosis Differential Diagnoses

Diagnostic Considerations

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