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Retroperitoneal Fibrosis Treatment & Management

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

Medical Care

Optimal care in patients with retroperitoneal fibrosis (RPF) requires an integrated approach of surgical and nonsurgical therapies. The aims of management are to preserve renal function, to prevent other organ involvement, to exclude malignancy, and to relieve symptoms.

The literature reports no consensus on the appropriate management of patients with retroperitoneal fibrosis because no controlled therapeutic trials have been performed. Furthermore, successful outcome has occasionally been reported with conservative therapy.[5, 6] In 2002, Pistolese et al reported partial or complete regression of retroperitoneal fibrosis associated with inflammatory aortic aneurysm after surgery.[13] The treatment of retroperitoneal fibrosis depends on the stage of the disease at diagnosis, as depicted in the image below.

Management algorithm of retroperitoneal fibrosis. Management algorithm of retroperitoneal fibrosis.

Empirical therapy includes corticosteroids, tamoxifen, and azathioprine; experimental therapy includes azathioprine, cyclophosphamide, mycophenolate-mofetil, cyclosporin, medroxyprogesterone acetate, and progesterone. Glucocorticoids and azathioprine are most useful in patients with signs of inflammation (eg, raised erythrocyte sedimentation rate [ESR] or C-reactive protein level, increased white blood cell count, and positive antinuclear antibody [ANA] results).

Corticosteroids

In 1958, Ross and Tinckler first reported the use of corticosteroids in the treatment of retroperitoneal fibrosis. The beneficial effect is thought to be due to anti-inflammatory action and the ability to inhibit fibrotic tissue maturation.

A pooled analysis of nonmalignant retroperitoneal fibrosis treated with steroids revealed a satisfactory outcome. In 2002, van Bommel analyzed 147 patients and noted good results in 122 patients (83%) and recurrence in 55 patients (16%). Most recurrences were noted within 12 months, and some responded to reintroduction of steroid.[44] Despite their proven success, using steroids as a first-line therapy in retroperitoneal fibrosis remains controversial because many clinicians believe that multiple deep biopsies are still essential to exclude malignancy.

A standard protocol is prednisolone at 40-60 mg/d tapered to 10 mg/d within 2-3 months and discontinued after 12-24 months. Timely dose reductions and cessation are important because of the adverse effects associated with long-term steroid use.

In 1994, Harreby et al used methylprednisolone pulse therapy (MPPT) at 1 g/d IV for 3 days along with azathioprine or penicillamine. This therapy was used in 11 cases of retroperitoneal fibrosis with ureteric obstruction following initial insertion of ureteral stents. The treatment was successful in 7 patients but only moderately effective in 4 patients. The combination of glucocorticoid and azathioprine is most useful in patients with signs of inflammation (raised ESR, positive ANA results, positive PET findings).[45]

Steroids can be used in combination with surgery. In one study, concomitant use of steroids with surgery reduced the rate of ureteric restenosis from 48% to 10%.[46] However, response may vary, and an unacceptably high dose of steroid may be required to control retroperitoneal fibrosis.

Long-term corticosteroid treatment can cause an array of adverse effects, including the following:

Tamoxifen

In 1991, Clarke et al were the first to use tamoxifen, a nonsteroidal antiestrogen, in the treatment of retroperitoneal fibrosis.[47] Clinicians have subsequently reported successful treatment with tamoxifen. Various authors have used tamoxifen with a variable protocol (10-40 mg for 6 mo to 3 y).

Its mechanism of action is not entirely clear, and different hypotheses have been proposed. Tamoxifen increases the synthesis and secretion of transforming growth factor–beta (TGF-β), an inhibitory growth factor, by human fetal fibroblast in vitro. In retroperitoneal fibrosis, fibroblast and immune cells in the inflammatory mass may increase their secretion of TGF-β, which may then decrease the size of the fibrous plaque.[48] Other possible mechanisms of action include inhibition of protein kinase C, reduction of epidermal growth factor production, inhibition of calmodulin, and blockage of growth-promoting histaminelike receptor.[49, 50]

A retrospective study by van der Bilt et al of initial treatment in 118 patients with idiopathic retroperitoneal fibrosis concluded that corticosteroids are superior to tamoxifen as monotherapy. In patients who respond to initial therapy with either medication, however, those treated with tamoxifen were less likely to experience relapse.[26]

Amelioration of symptoms after treatment initiation occurred in 2 weeks with steroids versus 4 weeks with tamoxifen (P <0.01), and mass regression at first follow-up CT scan was observed in 84.0% of steroid-treated patients versus 68.3% of those receiving tamoxifen (P = 0.05). Recurrence rate after successful initial treatment was 62.5% with steroids versus 21.4% with tamoxifen (P <0.01).[26]

Corticosteroids also proved superior to tamoxifen for maintaining remission of idiopathic retroperitoneal fibrosis, in a randomized, open-label, controlled trial in 40 patients (18-85 y) who had achieved remission with prednisone. Vaglio et al compared tapered prednisone (initial dose 0.5 mg/kg daily) with fixed-dose tamoxifen (0.5 mg/kg daily), both given for 8 months, and found that after 18 months, the estimated cumulative relapse probability was 17% with prednisone and 50% with tamoxifen (P=0.0372). However, cushingoid changes and grade 2 hypercholesterolemia were more common with prednisone.[51]

Compared with steroids, the adverse effect profile of tamoxifen is low; thus, clinicians consider tamoxifen a reasonable treatment option. However, the adverse effects of tamoxifen, especially an increased risk of thromboembolism and ovarian cancer, should be carefully considered for each patient.

Mycophenolate mofetil

To block the proliferation of T cells and B cells, a combination of mycophenolate mofetil (MMF) and steroid appears to be a promising option.

Swartz et al (2008) reported their experience with high-dose corticosteroid and MMF in 21 patients. They used prednisone 60 mg or 120 mg qid for 3-6 months and tapered the dose upon clinical improvement. In addition, steroid-sparing therapy with MMF (1000 mg bid) was recommended, with discontinuation after 6-12 months of therapy.[52]

Adler et al (2008) observed radiologic regression following therapy with MMF 2 g/d and prednisolone 1 mg/kg in 9 patients. Therapy led to removal of the ureteral catheter in 5 of 7 patients. Complications of MMF therapy included recurrent urinary infections and upper gastrointestinal disturbance.[53]

Scheel et al (2011) reported benefit in a prospective case series of 28 patients treated with MMF (1000 mg bid) and Prednisone, 40 mg/d, tapered over 6 months, for a mean of 24.3 months. In all patients, systemic symptoms resolved, and elevated ESR and serum creatinine level and decreased hemoglobin level normalized. Periaortic mass decreased by 25% or more in 89% of patients. Disease recurred in two patients.[54]

Azathioprine

Azathioprine has been used when steroid therapy has failed and as a steroid-sparing drug. Cogan and Fastrez used a 6-week course of azathioprine (150 mg/d) in a patient whose condition recurred soon after prednisone treatment was discontinued. They observed a significant response with azathioprine.[55] McDougal and MacDonell reported successful outcome in combination with prednisolone in a 14-year-old girl.[24]

Experimental therapy

More recently, immunosuppressive agents such as azathioprine, cyclophosphamide, MMF, methotrexate, and cyclosporin have been used to treat retroperitoneal fibrosis. Future steroid-sparing options could include anti-inflammatory drugs such as tumor necrosis factor-alpha (TNF-a) inhibitors and anti-CD20 drugs.[24, 56, 55, 57, 58]

Medroxyprogesterone acetate

In vitro, medroxyprogesterone acetate inhibits fibroblastic proliferation. Use of progesterone and medroxyprogesterone acetate as an alternative treatment has been reported with successful outcome.[59, 60]

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Surgical Care

See the list below:

  • Temporizing maneuvers in the form of percutaneous nephrostomy or ureteral stenting are recommended in the presence of obstructive uropathy.
  • Primary management of retroperitoneal fibrosis consists of open biopsy, ureterolysis, and lateral/intraperitoneal transposition or omental wrapping of the involved ureter, as depicted in the image below.
    Postureterolysis intravenous urogram demonstrates Postureterolysis intravenous urogram demonstrates lateral displacement of both ureters and a double J stent on the right side.
  • Open ureterolysis, although effective in 90% of patients, is associated with significant morbidity (60%) and mortality (9%) rates. [61, 62]
  • In recent decades, the use of laparoscopic surgery has expanded to include complex ablative and reconstructive procedures. Kavoussi first described laparoscopic ureterolysis for retroperitoneal fibrosis in 1992. [63] Since then, a few authors have reported successful laparoscopic ureterolysis with a more rapid recovery and a shorter hospital stay. Although the success rate is no better than that of open ureterolysis, the laparoscopic technique has the advantage of reducing mean hospital stay, use of analgesia, convalescence period, and morbidity. [63, 64, 65]
  • Styn et al compared open ureterolysis (12 patients) with laparoscopic ureterolysis (13 patients) and reported a significantly shorter hospital stay (open 5.9 d vs laparoscopic 2.1 d; p=.004). [66] The complication rates did not differ between the groups. The success rate was 87.5% after open ureterolysis and 93.8% after laparoscopic ureterolysis (p=1).
  • More recently, with the advancement of technology, cases of endourologic treatment of retroperitoneal fibrosis via percutaneous balloon dilatation or endoscopic incision, dilatation, and permanent wall stent have been reported, with varying results. [7]
  • Long-term ureteral stenting is a reasonable approach in high-risk and elderly patients. Ureteral stenting may be placed on a long-term basis (months to years) in order to bypass ureteral obstruction. Short-term stenting (weeks to months) may be used as an adjunct to open surgical procedures.
  • Other newer innovative surgical techniques have been described, such as ureterolysis and wrapping with Gore-Tex (GSM, WL Gore & Associates, Flagstaff, Ariz), excision of the ureter and reanastomosis, posterior preperitoneal flap, and renal autotransplantation. [8]
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Consultations

Patients with renal failure should be referred to a nephrologist early in the course of their disease and have continued nephrologic follow-up.

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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 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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