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primary localized amyloidosis of the ureter
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nephrology image
& 2015 International Society of Nephrology Kidney International (2015) 87, 1267–1268; doi:10.1038/ki.2014.313
Nephrology image/primary localized amyloidosis of the ureter Vesna Jurcˇic´1, Robert Kordicˇ2 and Jera Jeruc1 1
Faculty of Medicine, Institute of Pathology, University of Ljubljana, Ljubljana, Slovenia and 2Department of Urology, University Medical Centre Ljubljana, Ljubljana, Slovenia
Correspondence: Vesna Jurcˇic´, Faculty of Medicine, Institute of Pathology, University of Ljubljana, Korytkova 2, SI-1000 Ljubljana, Slovenia. E-mail: [email protected]
Figure 1 | Retrograde pyelogram shows tapering and marked narrowing of the distal and dilatation of the proximal left ureter, pyelon, and renal calices (ureterohydronephrosis). Note irregular contours of the entire ureteral mucosa (amyloidosis involved also the proximal ureter and ureteropelvic junction).
Figure 2 | Photomicrograph shows abundant amyloid deposits in Congo red stain (original magnification 100).
A 67-year-old woman with a history of recurrent urinary tract infections was admitted for investigation of intermittent gross hematuria and left flank pain. All blood tests were normal. Urinalysis revealed hematuria. Urine cultures were negative. Cystoscopy was unremarkable. Urine cytology was inconclusive; there were small clusters of urothelial cells showing mild atypia. Retrograde pyelography (Figure 1) and computed tomography urography were performed, showing hydroureteronephrosis above a tumor-like narrowing in the
lower part of the ureter. A diagnosis of ureteral malignancy was made. She underwent left laparoscopic nephrectomy. Microscopic examination revealed chronic ureteritis and extensive deposition of red-orange amyloid deposits in Congo red staining (Figure 2) that displayed typical yellow-green birefringence under polarized light. Immunohistochemistry was positive for lambda light chains. Immunoelectrophoresis of serum and urine, Bence-Jones’s protein test, levels of immunoglobulins, and light chains were all normal.
Kidney International (2015) 87, 1267–1268
1267
nephrology image
Results of bone marrow biopsy and biopsy of subcutaneous fat tissue were normal. With these results systemic amyloidosis was excluded. Localized amyloidosis of the ureter is a rare disorder with fewer than 200 cases reported. It is almost exclusively of amyloid light chain type. The exact cause is not known; it has been hypothesized that chronic and recurrent inflammation with a local production of immunoglobulins has an important role in the pathogenesis. It often manifests with hematuria and signs secondary to ureteral obstruction.
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V Jurcˇic´ et al.: Primary localized amyloidosis of the ureter
Typical imaging findings include focal or diffuse areas of wall thickening, calcifications, intraureteral filling defects, and irregular ureteral narrowing and stricture, which results in hydronephrosis. These findings are indistinguishable from those of urothelial carcinoma. Calculus, tuberculosis, stricture, and endometriosis are also included in the differential diagnosis. The diagnosis is therefore based on histopathological findings. Preoperative biopsy is recommended to avoid overtreatment, resulting in loss of healthy kidney. The prognosis is excellent in most cases, although disease recurrence is possible.
Kidney International (2015) 87, 1267–1268
nephrology image
& 2015 International Society of Nephrology Kidney International (2015) 87, 1267–1268; doi:10.1038/ki.2014.313
Nephrology image/primary localized amyloidosis of the ureter Vesna Jurcˇic´1, Robert Kordicˇ2 and Jera Jeruc1 1
Faculty of Medicine, Institute of Pathology, University of Ljubljana, Ljubljana, Slovenia and 2Department of Urology, University Medical Centre Ljubljana, Ljubljana, Slovenia
Correspondence: Vesna Jurcˇic´, Faculty of Medicine, Institute of Pathology, University of Ljubljana, Korytkova 2, SI-1000 Ljubljana, Slovenia. E-mail: [email protected]
Figure 1 | Retrograde pyelogram shows tapering and marked narrowing of the distal and dilatation of the proximal left ureter, pyelon, and renal calices (ureterohydronephrosis). Note irregular contours of the entire ureteral mucosa (amyloidosis involved also the proximal ureter and ureteropelvic junction).
Figure 2 | Photomicrograph shows abundant amyloid deposits in Congo red stain (original magnification 100).
A 67-year-old woman with a history of recurrent urinary tract infections was admitted for investigation of intermittent gross hematuria and left flank pain. All blood tests were normal. Urinalysis revealed hematuria. Urine cultures were negative. Cystoscopy was unremarkable. Urine cytology was inconclusive; there were small clusters of urothelial cells showing mild atypia. Retrograde pyelography (Figure 1) and computed tomography urography were performed, showing hydroureteronephrosis above a tumor-like narrowing in the
lower part of the ureter. A diagnosis of ureteral malignancy was made. She underwent left laparoscopic nephrectomy. Microscopic examination revealed chronic ureteritis and extensive deposition of red-orange amyloid deposits in Congo red staining (Figure 2) that displayed typical yellow-green birefringence under polarized light. Immunohistochemistry was positive for lambda light chains. Immunoelectrophoresis of serum and urine, Bence-Jones’s protein test, levels of immunoglobulins, and light chains were all normal.
Kidney International (2015) 87, 1267–1268
1267
nephrology image
Results of bone marrow biopsy and biopsy of subcutaneous fat tissue were normal. With these results systemic amyloidosis was excluded. Localized amyloidosis of the ureter is a rare disorder with fewer than 200 cases reported. It is almost exclusively of amyloid light chain type. The exact cause is not known; it has been hypothesized that chronic and recurrent inflammation with a local production of immunoglobulins has an important role in the pathogenesis. It often manifests with hematuria and signs secondary to ureteral obstruction.
1268
V Jurcˇic´ et al.: Primary localized amyloidosis of the ureter
Typical imaging findings include focal or diffuse areas of wall thickening, calcifications, intraureteral filling defects, and irregular ureteral narrowing and stricture, which results in hydronephrosis. These findings are indistinguishable from those of urothelial carcinoma. Calculus, tuberculosis, stricture, and endometriosis are also included in the differential diagnosis. The diagnosis is therefore based on histopathological findings. Preoperative biopsy is recommended to avoid overtreatment, resulting in loss of healthy kidney. The prognosis is excellent in most cases, although disease recurrence is possible.
Kidney International (2015) 87, 1267–1268