J Korean Soc Radiol.  2016 Jun;74(6):412-416. 10.3348/jksr.2016.74.6.412.

Idiopathic Retroperitoneal Fibrosis Involving a Unilateral Renal Sinus: A Case Report and Literature Review

• Lee SB
• Yoon JH
• Kim SH
• Lee Y
• Kim SJ
• Lim YJ
• Jung HK
• Lee JS

• KMID: 2164821
• DOI: http://doi.org/10.3348/jksr.2016.74.6.412

Abstract

Idiopathic retroperitoneal fibrosis (RPF) is a rare disease entity and its etiology is uncertain. We report two similar cases which showed an uncommon presentation of idiopathic RPF. A 66-year-old woman and an 80-year-old man presented with incidental findings of left renal pelvic mass-like lesions. Computed tomography revealed a soft tissue density mass replacing the left renal pelvis, which was suspicious for renal pelvic cancer, and the diagnosis of idiopathic RPF was surgically confirmed. To the best of our knowledge, a few cases of idiopathic RPF presenting with features of a localized unilateral renal pelvic mass mimicking renal pelvic cancer have been reported.

MeSH Terms

Aged
Aged, 80 and over
Diagnosis
Female
Humans
Incidental Findings
Kidney
Kidney Pelvis
Pelvic Neoplasms
Rare Diseases
Retroperitoneal Fibrosis*
Tomography, X-Ray Computed

Figure

• 

  Fig. 1 A 66-year-old woman who underwent left nephrectomy due to idiopathic retroperitoneal fibrosis mimicking urothelial carcinoma. A, B. Contrast enhanced CT scan axial image in the nephrographic phase (A), and coronal image in the corticomedullary phase (B) show a homogeneously enhancing soft tissue mass (arrows) replacing the left renal pelvis. C. Microscopic examination of the resected kidney specimen shows diffuse fibrosis (black arrow) and chronic inflammation with myofibroblast proliferation (arrowhead) and lymphoplasma cell infiltrates (white arrow) mainly located in the renal sinus fat tissue (hematoxylin and eosin stain, × 40).

• 

  Fig. 2 An 80-year-old man with the incidental finding of a left renal pelvic mass-like lesion. A, B. Contrast enhanced CT scan axial images in the nephrographic phase (A), and coronal image in the corticomedullary phase (B) show an enhancing soft tissue mass (arrows) in the left renal pelvis and sinus with mild pelvic dilatation. Periureteric fat infiltration in the left proximal ureter without definite wall thickening is noted. C. Retrograde pyelography shows suspected luminal narrowing of the renal pelvis and mild calyectasia, but there is no definite abnormal filling defect. D. Microscopic examination of the resected kidney specimen shows diffuse stromal fibrosis (black arrow) with lymphoplasma cell infiltration and reactive lymphoid follicle formation (white arrow), which is compatible with retroperitoneal fibrosis, mainly located in the renal sinus fat tissue (arrowhead) (hematoxylin and eosin stain, × 40).

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