- Email: [email protected]
Secondary Amyloidosis as an Unusual Cause of Ureteral Obstruction
0022-5347/04/1711-0355/0 THE JOURNAL OF UROLOGY® Copyright © 2004 by AMERICAN UROLOGICAL ASSOCIATION
Vol. 171, 355–356, January 2004 Printed in U.S.A.
DOI: 10.1097/01.ju.0000098862.45327.ff
SECONDARY AMYLOIDOSIS AS AN UNUSUAL CAUSE OF URETERAL OBSTRUCTION DANIEL W. CARLSON, DAVID R. FINGER
AND
CHRISTINA M. BELNAP
From Tripler Army Medical Center, Honolulu, Hawaii KEY WORDS: amyloidosis, ureter
Localized amyloidosis of the urogenital tract is a rarely described cause of ureteral obstruction and renal failure. Most previously described cases involve primary amyloidosis. We report a case of unilateral ureteral obstruction caused by localized secondary amyloidosis, which to our knowledge has only been described once previously.1 CASE REPORT
A 53-year-old female was incidentally found to have unilateral hydronephrosis and hydroureter on computerized tomography of the chest. Computerized pyelography demonstrated a left distal ureteral mass as well as hydronephrosis and hydroureter (fig. 1). The patient underwent ureteroscopy, biopsy and stenting. Biopsies revealed acute and chronic inflammation but no definitive diagnosis. Ureteral brushings and urine cytology were negative. Despite stenting, unilateral renal function did not improve, as the function of the affected side remained less than 10% on renal scintigraphy. Since no definitive diagnosis was made and the renal dysfunction persisted, laparoscopic nephroureterectomy was performed. Histopathological analysis of the ureteral mass showed secondary amyloidosis (fig. 2). Further testing demonstrated mild polyclonal gammopathy with a normal C-reactive protein level. Evaluation for systemic amyloidosis was negative, to include abdominal fat pad biopsy and urine protein electrophoresis. The patient was empirically started on colchicine and the polyclonal gammopathy resolved. She remains asymptomatic 2 years later with normal renal function. DISCUSSION
Amyloidosis is characterized by extracellular tissue deposition of proteins, which can be primary (usually the result of a plasma cell dyscrasia such as multiple my-
Accepted for publication August 8, 2003.
FIG. 2. Sections of ureter reveal extracellular amyloid deposits, which appear as amorphous, eosinophilic material in subepithelial connective tissue. Reduced from ⫻40.
eloma) or secondary (caused by deposition of acute phase proteins such as serum amyloid A). Historically, secondary amyloidosis was most commonly associated with chronic infectious diseases such as tuberculosis but it is currently seen more often with chronic inflammatory diseases such as rheumatoid arthritis.2 Immunohistochemical staining has revealed that most cases of localized urogenital amyloidosis are of the primary type, with and light chains. Our patient had localized urogenital amyloidosis of the secondary subtype, which to our knowledge has only been reported once previously.1 In that instance the patient presented with unilateral distal ureteral obstruction with hydronephrosis and gross hematuria, and underwent ureterectomy and reimplantation. The pathophysiology of localized secondary amyloidosis is unknown, but it has been shown that localized production of serum amyloid can be induced by interleukin-1 and 6, cytokines that are typically increased when inflammation is present.3 Treatment for urogenital amyloidosis has historically included either partial ureterectomy or nephroureterectomy, as the definitive diagnosis is often unknown before resection. After diagnosis evaluation for systemic amyloidosis is recommended. Additionally, patients with primary amyloidosis should be evaluated for a plasma cell dyscrasia, while a systemic inflammatory condition should be sought in those with secondary amyloidosis. Localized urogenital amyloidosis remains a rare etiology of ureteral obstruction and unilateral renal failure. Most cases involve the distal ureter and are associated with primary amyloid protein deposition. Our patient had secondary amyloid deposition, which appears to represent an even more uncommon etiology of ureteral obstruction. Surgical resection is often done to establish a definitive diagnosis, although
FIG. 1. Abdominal computerized tomography demonstrates left hydronephrosis and hydroureter. 355
356
AMYLOIDOSIS CAUSING URETERAL OBSTRUCTION
the ideal systemic evaluation and treatment recommendations are less certain. REFERENCES
1. Fugita, E. O., DeLatorre, C. G. and Kavoussi, L. R.: Primary localized amyloidosis of the ureter. Urology, 58: 281, 2001
2. Falk, R. H., Comenzo, R. L. and Skinner, M.: The systemic amyloidoses. N Engl J Med, 337: 898, 1997 3. Ray, A., Schatten, H. and Ray, B. K.: Activation of Sp1 and its functional co-operation with serum amyloid A-activating sequence binding factor in synoviocyte cells trigger synergistic action of interleukin-1 and interleukin-6 in serum amyloid A gene expression. J Biol Chem, 274: 4300, 1999
Vol. 171, 355–356, January 2004 Printed in U.S.A.
DOI: 10.1097/01.ju.0000098862.45327.ff
SECONDARY AMYLOIDOSIS AS AN UNUSUAL CAUSE OF URETERAL OBSTRUCTION DANIEL W. CARLSON, DAVID R. FINGER
AND
CHRISTINA M. BELNAP
From Tripler Army Medical Center, Honolulu, Hawaii KEY WORDS: amyloidosis, ureter
Localized amyloidosis of the urogenital tract is a rarely described cause of ureteral obstruction and renal failure. Most previously described cases involve primary amyloidosis. We report a case of unilateral ureteral obstruction caused by localized secondary amyloidosis, which to our knowledge has only been described once previously.1 CASE REPORT
A 53-year-old female was incidentally found to have unilateral hydronephrosis and hydroureter on computerized tomography of the chest. Computerized pyelography demonstrated a left distal ureteral mass as well as hydronephrosis and hydroureter (fig. 1). The patient underwent ureteroscopy, biopsy and stenting. Biopsies revealed acute and chronic inflammation but no definitive diagnosis. Ureteral brushings and urine cytology were negative. Despite stenting, unilateral renal function did not improve, as the function of the affected side remained less than 10% on renal scintigraphy. Since no definitive diagnosis was made and the renal dysfunction persisted, laparoscopic nephroureterectomy was performed. Histopathological analysis of the ureteral mass showed secondary amyloidosis (fig. 2). Further testing demonstrated mild polyclonal gammopathy with a normal C-reactive protein level. Evaluation for systemic amyloidosis was negative, to include abdominal fat pad biopsy and urine protein electrophoresis. The patient was empirically started on colchicine and the polyclonal gammopathy resolved. She remains asymptomatic 2 years later with normal renal function. DISCUSSION
Amyloidosis is characterized by extracellular tissue deposition of proteins, which can be primary (usually the result of a plasma cell dyscrasia such as multiple my-
Accepted for publication August 8, 2003.
FIG. 2. Sections of ureter reveal extracellular amyloid deposits, which appear as amorphous, eosinophilic material in subepithelial connective tissue. Reduced from ⫻40.
eloma) or secondary (caused by deposition of acute phase proteins such as serum amyloid A). Historically, secondary amyloidosis was most commonly associated with chronic infectious diseases such as tuberculosis but it is currently seen more often with chronic inflammatory diseases such as rheumatoid arthritis.2 Immunohistochemical staining has revealed that most cases of localized urogenital amyloidosis are of the primary type, with and light chains. Our patient had localized urogenital amyloidosis of the secondary subtype, which to our knowledge has only been reported once previously.1 In that instance the patient presented with unilateral distal ureteral obstruction with hydronephrosis and gross hematuria, and underwent ureterectomy and reimplantation. The pathophysiology of localized secondary amyloidosis is unknown, but it has been shown that localized production of serum amyloid can be induced by interleukin-1 and 6, cytokines that are typically increased when inflammation is present.3 Treatment for urogenital amyloidosis has historically included either partial ureterectomy or nephroureterectomy, as the definitive diagnosis is often unknown before resection. After diagnosis evaluation for systemic amyloidosis is recommended. Additionally, patients with primary amyloidosis should be evaluated for a plasma cell dyscrasia, while a systemic inflammatory condition should be sought in those with secondary amyloidosis. Localized urogenital amyloidosis remains a rare etiology of ureteral obstruction and unilateral renal failure. Most cases involve the distal ureter and are associated with primary amyloid protein deposition. Our patient had secondary amyloid deposition, which appears to represent an even more uncommon etiology of ureteral obstruction. Surgical resection is often done to establish a definitive diagnosis, although
FIG. 1. Abdominal computerized tomography demonstrates left hydronephrosis and hydroureter. 355
356
AMYLOIDOSIS CAUSING URETERAL OBSTRUCTION
the ideal systemic evaluation and treatment recommendations are less certain. REFERENCES
1. Fugita, E. O., DeLatorre, C. G. and Kavoussi, L. R.: Primary localized amyloidosis of the ureter. Urology, 58: 281, 2001
2. Falk, R. H., Comenzo, R. L. and Skinner, M.: The systemic amyloidoses. N Engl J Med, 337: 898, 1997 3. Ray, A., Schatten, H. and Ray, B. K.: Activation of Sp1 and its functional co-operation with serum amyloid A-activating sequence binding factor in synoviocyte cells trigger synergistic action of interleukin-1 and interleukin-6 in serum amyloid A gene expression. J Biol Chem, 274: 4300, 1999