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Bilateral Ureteral Primary Amyloidosis
THE JorRNAL OF UROLOGY
Vol. 02, No. 4 October 1064 Copyright© 1064 by The Williams & Wilkins Co. Printed in U.S.A.
BILATERAL URETERAL PRIMARY AMYLOIDOSIS HJALMAR W. JOHNSON
AND
G. JOHN ANKENMAN
From the Urology Division, Department of Surgery, University of British Colwnbia, Yancouver, British Columbia, Canada
Primary amyloidosis is a relatively rare disease characterized by deposition of a hyaline glycoprotein substance in various organs of the body. 1 The organs involved include spleen, heart and blood vessels, as well as kidney, ureter, bladder, prostate, seminal vesicles and urethra. 2- 6 The
various conditions including chronic inflammation and multiple rn.yeloma-the so-called secondary amyloidosis. Unilateral ureteral primary amyloidosis has been reported. 7 • 8 This is a case report of primary amyloidosis with bilateral ureteral involvement.
Fm. 1. A, excretory urogram shows right ureteral obstruction. B, excretory urogram, 40-minute film demonstrates degree of right ureteral obstruction.
disease is a manifestation of deranged protein metabolism of unknown etiology. There is no chern.ical or histological difference in the amyloicl of the primary disease and that associated with Accepted for publication January 24, 1964. 1 Symrrnrs, W. S. C. · Primary amyloidosis. J. Clin. Path., 9: 187, 1956. 2 McDonald, J. H. and Heckel, N. J.: Primary amyloidosis of the lower genitourinary tract. J. Urol., 75: 122, 1956. 3 Kenzel, R. C., Harrison, E.G., Jr. and Utz, D. C.: Primary amyloidosis of the bladder. J. Urol., 85: 785, 1961.
4 Fraser, J. G. and Kaye, 1VI.: Renal amyloidosis. Canad. Med. Assoc . .J., 85: 967, 1961. 5 Nagel, R.: Localized amyloidosis of the bladder . .J. Urol., 88: 56, 1962. " Goldman, H.: Amyloidosis of seminal vesicles and vas clefercns. Arch. Path., 75: 94, 1963.
CASE REPORT
A 17-year-old male prison inmate was first investigated at the Vancouver General Hospital in September 1961, because of complaints of dull, aching, right flank pain and gross total, painless henmturia. Physical exan1ination revealed a young man in good health except for moderate right flank tenderness on deep palpation. The laboratory findings were macroscopic hematuria; sterile urine on culture; hemoglobin, 13.0 gm. per 100 cc blood; white blood cell count, 7,050 per 7 Gilbert, L. W. and McDonald, ,T. R.: Amyloidosis of renal pelvis and ureter. J. Urol., 68: 137,
1952.
8 Andreas, B. F. and Oosting, 1VI.: Primary amyloidosis of the ureters . .J. Urol., 79: 929, 1958.
27.5
276
JOHNSON AND ANKENMAN
Fm. 2. Microscopic appearance of right ureteral wall. Methyl violet stain, XllO.
Fm. 3. Excretory urogram shows left ureteral obstruction.
cu. mm. blood; and erythrocyte sedimentation rate (Westergren), 4 mm. in 1 hour. Roentgenographic examination of the chest was normal. The excretory urogram (fig. 1, A) showed right hydronephrosis and hydroureter extending down to the mid-pelvic portion of the ureter (fig. 1, B). At cystoscopy, performed under general anesthesia, no infravesical obstruction was present. Diffuse hemorrhagic cystitis was present and catheterization of the right ureter beyond 3 mm. was not possible. The left collecting system was normal. Indigo carmine injected intravenously appeared in dilute concentration from the right ureteral orifice in 10 minutes and appeared in good concentration from the left orifice in 5 minutes. Definitive treatment was deferred pending investigation of the patient for tuberculosis. Cultures and guinea pig inoculation were negative. The patient was re-admitted 2 months later. Physical examination and laboratory and roentgenographic investigation were essentially unchanged from the previous admission. Exploration was performed through a midline lower abdominal incision. The ureter was found to be dilated down to the mid-pelvic portion where there was a thickening of the wall extending down to the bladder. As the diagnosis was in
Fm. 4. Microscopic appearance of left ureteral wall. H & E Xl20.
BILATERAL URETERAL PRIMARY AMYLOIDOSIS
doubt, the lower ureter and a cuff of bladder were resected and the proxi1nal ureter was brought out as a cutaneous ureterostomy. The resected segment of ureter was diffusely hemorrhagic and firm and the wall measured up to 1 cn1. in thickness. The histologic appearance was that of infiltration throughout all layers of the ureteral wall of a homogeneous hyaline material which proved to be amyloid when special stains (methyl violet and fluorescent teclmiques) were used (fig. 2). In view of the benign nature of the lesion, a definitive ureteroneocystostomy was later performed using a tube flap of bladder wall. Complete investigation failed to detect any evidence of systemic disease; therefore, the ureteral lesion was considered primary amyloidosis. The patient was well for 1 year at which time he was readmitted to the hospital because of gross hematuria, accompanied by left flank pain. The only abnormal physical findings were left flank tenderness and grossly bloody urine. Excretory urography revealed left hydronephrosis and hydrometer (fig. 3). At cystoscopy the bladder mucosa was edematous and there were hemorrhagic areas around the left ureteral orifice. A catheter would not pass up the left ureteral orifice and indigo carmine injected intravenously did not appear from the left side. Through a
277
midline incision the left lower ureter was subsequently explored. The obstruction was due to diffuse edematous, friable thickening involving the wall of the terminal 2 cm. and the intramural ureter, similar to the gross appearance previously encountered on the right side. This segment of ureter with a cuff of bladder wall was resected and a ureteroneocystostomy, using the PolitanoLeadbetter technique, 9 was performed. The histologic diagnosis was again amyloidosis of ureteral wall (fig. 4). Complete investigation was again undertaken and no evidence of systern.ic disease was found. The patient is well 9 months later. However, it is possible that residual disease remains in the bladder base or seminal vesicles and long-term followup evaluation is indicated. SUMMARY
There are 27 reported cases of primary amy loidosis involving the ureter, to which is added the present case-the only one with bilateral ureteral involvement. The urological aspect of primary amyloidosis is of interest and should be considered in the diagnosis and management of ureteral and bladder tumors. 9 Politano, V. A. and Leadbetter, W. F.: An operative technique for correction of vesicoureteral reflux. J. Urol., 79: 932, 1958.
Vol. 02, No. 4 October 1064 Copyright© 1064 by The Williams & Wilkins Co. Printed in U.S.A.
BILATERAL URETERAL PRIMARY AMYLOIDOSIS HJALMAR W. JOHNSON
AND
G. JOHN ANKENMAN
From the Urology Division, Department of Surgery, University of British Colwnbia, Yancouver, British Columbia, Canada
Primary amyloidosis is a relatively rare disease characterized by deposition of a hyaline glycoprotein substance in various organs of the body. 1 The organs involved include spleen, heart and blood vessels, as well as kidney, ureter, bladder, prostate, seminal vesicles and urethra. 2- 6 The
various conditions including chronic inflammation and multiple rn.yeloma-the so-called secondary amyloidosis. Unilateral ureteral primary amyloidosis has been reported. 7 • 8 This is a case report of primary amyloidosis with bilateral ureteral involvement.
Fm. 1. A, excretory urogram shows right ureteral obstruction. B, excretory urogram, 40-minute film demonstrates degree of right ureteral obstruction.
disease is a manifestation of deranged protein metabolism of unknown etiology. There is no chern.ical or histological difference in the amyloicl of the primary disease and that associated with Accepted for publication January 24, 1964. 1 Symrrnrs, W. S. C. · Primary amyloidosis. J. Clin. Path., 9: 187, 1956. 2 McDonald, J. H. and Heckel, N. J.: Primary amyloidosis of the lower genitourinary tract. J. Urol., 75: 122, 1956. 3 Kenzel, R. C., Harrison, E.G., Jr. and Utz, D. C.: Primary amyloidosis of the bladder. J. Urol., 85: 785, 1961.
4 Fraser, J. G. and Kaye, 1VI.: Renal amyloidosis. Canad. Med. Assoc . .J., 85: 967, 1961. 5 Nagel, R.: Localized amyloidosis of the bladder . .J. Urol., 88: 56, 1962. " Goldman, H.: Amyloidosis of seminal vesicles and vas clefercns. Arch. Path., 75: 94, 1963.
CASE REPORT
A 17-year-old male prison inmate was first investigated at the Vancouver General Hospital in September 1961, because of complaints of dull, aching, right flank pain and gross total, painless henmturia. Physical exan1ination revealed a young man in good health except for moderate right flank tenderness on deep palpation. The laboratory findings were macroscopic hematuria; sterile urine on culture; hemoglobin, 13.0 gm. per 100 cc blood; white blood cell count, 7,050 per 7 Gilbert, L. W. and McDonald, ,T. R.: Amyloidosis of renal pelvis and ureter. J. Urol., 68: 137,
1952.
8 Andreas, B. F. and Oosting, 1VI.: Primary amyloidosis of the ureters . .J. Urol., 79: 929, 1958.
27.5
276
JOHNSON AND ANKENMAN
Fm. 2. Microscopic appearance of right ureteral wall. Methyl violet stain, XllO.
Fm. 3. Excretory urogram shows left ureteral obstruction.
cu. mm. blood; and erythrocyte sedimentation rate (Westergren), 4 mm. in 1 hour. Roentgenographic examination of the chest was normal. The excretory urogram (fig. 1, A) showed right hydronephrosis and hydroureter extending down to the mid-pelvic portion of the ureter (fig. 1, B). At cystoscopy, performed under general anesthesia, no infravesical obstruction was present. Diffuse hemorrhagic cystitis was present and catheterization of the right ureter beyond 3 mm. was not possible. The left collecting system was normal. Indigo carmine injected intravenously appeared in dilute concentration from the right ureteral orifice in 10 minutes and appeared in good concentration from the left orifice in 5 minutes. Definitive treatment was deferred pending investigation of the patient for tuberculosis. Cultures and guinea pig inoculation were negative. The patient was re-admitted 2 months later. Physical examination and laboratory and roentgenographic investigation were essentially unchanged from the previous admission. Exploration was performed through a midline lower abdominal incision. The ureter was found to be dilated down to the mid-pelvic portion where there was a thickening of the wall extending down to the bladder. As the diagnosis was in
Fm. 4. Microscopic appearance of left ureteral wall. H & E Xl20.
BILATERAL URETERAL PRIMARY AMYLOIDOSIS
doubt, the lower ureter and a cuff of bladder were resected and the proxi1nal ureter was brought out as a cutaneous ureterostomy. The resected segment of ureter was diffusely hemorrhagic and firm and the wall measured up to 1 cn1. in thickness. The histologic appearance was that of infiltration throughout all layers of the ureteral wall of a homogeneous hyaline material which proved to be amyloid when special stains (methyl violet and fluorescent teclmiques) were used (fig. 2). In view of the benign nature of the lesion, a definitive ureteroneocystostomy was later performed using a tube flap of bladder wall. Complete investigation failed to detect any evidence of systemic disease; therefore, the ureteral lesion was considered primary amyloidosis. The patient was well for 1 year at which time he was readmitted to the hospital because of gross hematuria, accompanied by left flank pain. The only abnormal physical findings were left flank tenderness and grossly bloody urine. Excretory urography revealed left hydronephrosis and hydrometer (fig. 3). At cystoscopy the bladder mucosa was edematous and there were hemorrhagic areas around the left ureteral orifice. A catheter would not pass up the left ureteral orifice and indigo carmine injected intravenously did not appear from the left side. Through a
277
midline incision the left lower ureter was subsequently explored. The obstruction was due to diffuse edematous, friable thickening involving the wall of the terminal 2 cm. and the intramural ureter, similar to the gross appearance previously encountered on the right side. This segment of ureter with a cuff of bladder wall was resected and a ureteroneocystostomy, using the PolitanoLeadbetter technique, 9 was performed. The histologic diagnosis was again amyloidosis of ureteral wall (fig. 4). Complete investigation was again undertaken and no evidence of systern.ic disease was found. The patient is well 9 months later. However, it is possible that residual disease remains in the bladder base or seminal vesicles and long-term followup evaluation is indicated. SUMMARY
There are 27 reported cases of primary amy loidosis involving the ureter, to which is added the present case-the only one with bilateral ureteral involvement. The urological aspect of primary amyloidosis is of interest and should be considered in the diagnosis and management of ureteral and bladder tumors. 9 Politano, V. A. and Leadbetter, W. F.: An operative technique for correction of vesicoureteral reflux. J. Urol., 79: 932, 1958.