Investig Clin Urol.  2017 Sep;58(5):371-377. 10.4111/icu.2017.58.5.371.

Retroperitoneal fibrosis-clinical presentation and outcome analysis from urological perspective

• Jadhav KK
• Kumar V
• Punatar CB
• Joshi VS
• Sagade SN

• KMID: 2388055
• DOI: http://doi.org/10.4111/icu.2017.58.5.371

Abstract

PURPOSE
To study clinical presentation, laboratory results, imaging findings and treatment options and outcomes of retroperitoneal fibrosis (RPF). To determine whether it follows the same natural course and response to treatment in the Asian population as in the Western world.
MATERIALS AND METHODS
Medical records of patients diagnosed with RPF on imaging and histopathology between February 2010 and April 2016 were reviewed.
RESULTS
Of the 21 patients analyzed, mean age at presentation was 50.81 years. The male to female ratio was 0.9:1. Pain was most common presenting complaint (95.23% cases), almost 85% cases were idiopathic and rests were postradiation induced. The median creatinine level was 1.8 mg/dL. The mean erythrocyte sedimentation rate (ESR) was 53.2 mm/h. Hydronephrosis was present in all patients and 47.6% had atrophic kidneys. Diffuse retroperitoneal mass was present in 61.1%. Ureterolysis with lateralization, omental wrapping or gonadal pedicle wrap was done in 17 cases. Two patients underwent uretero-ureterostomy. One patient underwent ileal replacement of ureter, and one ileal conduit. Eighteen patients received concurrent medical treatment, 11 were given tamoxifen, 2 steroids (Prednisolone), and five were given both. Of the 20 patients with follow-up, 70% had complete symptomatic relief; ESR improvement was seen in 77.8%. Follow-up ultrasound showed resolved and decreased hydronephrosis in 20% and 55% respectively. One patient had treatment failure and 17.65% had disease recurrence.
CONCLUSIONS
RPF is a rare disease with varied presentation and outcomes. The male to female ratio may be equal in Asians and smoking could be lesser contributing factor. More Asian cohort studies are required to support same.

Keyword

Retroperitoneal fibrosis; Signs and symptoms; Treatment outcome; Ureteral obstruction

MeSH Terms

Asian Continental Ancestry Group
Blood Sedimentation
Cohort Studies
Creatinine
Female
Follow-Up Studies
Gonads
Humans
Hydronephrosis
Kidney
Male
Medical Records
Rare Diseases
Recurrence
Retroperitoneal Fibrosis
Smoke
Smoking
Steroids
Tamoxifen
Treatment Failure
Treatment Outcome
Ultrasonography
Ureter
Ureteral Obstruction
Urinary Diversion
Western World
Creatinine
Smoke
Steroids
Tamoxifen

Figure

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  Fig. 1 Intravenous urography showing medialization of left ureter with hydroureteronephrosis and poorly functioning right kidney.

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  Fig. 2 Pretreatment (A) and posttreatment (B) computed tomography scan showing retroperitoneal mass and improvement in function and excretion on right side.

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