Radiographic Exhibit
Localized Amyloidosis of the Ureter 1 King 1ak Lee, M.D., and 10ny M. Deeths, M.D. A case of localized amyloidosis of the distal ureter in a 51-year-old man is described. Pertinent features of previously reported cases are described, and the difficulty of distinguishing this entity from primary carcinoma of the ureter is discussed. INDEX TERMS:
Amyloidosis. Ureters, diseases. Ureters, neoplasms
Radiology 120:60, July 1976
of the urinary tract is relatively ~ncom mon, and localized involvement of the ureter IS even less frequent. Only 10 such cases have been reported (3, 4). We wish to add the following case. AMYLOIDOSIS
.K
CASE REPORT A 51-year-old white man was evaluated following the sudden onset of colicky left flank pain and hematuria. Physical examination revealed mild tenderness of the left costovertebral angle. Urinalysis showed more than 50 red blood cells and 2 white blood cells per high-power field, but no protein or bacteria. Routine laboratory tests were normal. Excretory urography demonstrated delayed function on the left and moderate ureteropyelocaliectasis. A stenotic distal ureteral segment measuring 1 em was seen 3 em above the ureterovesical junction with dilatation distal to it, a picture most consistent with neoplasm, although a benign stricture could not be excluded. At cystoscopy, the bladder and ureteral orifices were normal. Retrograde pyelography (Fig. 1) showed changes similar to those seen on the urogram. Cytological examination of brushings from the stenotic region was negative. At exploratory laparotomy, the constricted area was found to be rock-hard. The high suspicion of carcinoma and the technical difficulty of doing a partial ureteral resection in this case necessitated a left nephro-ureterectomy. Microscopic examination of the indurated area of the ureter revealed amyloid deposits beneath a denuded mucosa, with mild infiltration by lymphocytes and a few multinucleated giant cells. The arteries were surrounded by amyloid deposits. The kidney itself was normal.
Fig. 1. Retrograde pyelogram demonstrating proximal and distal dilatation, irregular shelving at the margins of the stricture, and the small caliber of the strictured region.
had the usual presenting symptoms of flank pain and hematuria, he did not have the insidious onset of symptoms characteristic of other reported cases. Although submucosal calcification is pathognomonic of primary amyloidosis of the renal pelvis and calyces (1), localized amyloid of the ureter has no distinctive appearance and is generally indistinguishable from strictures or from intrinsic or extrinsic neoplasm. Because of the nonspecificity of the radiographic findings, and because tumor is difficult to exclude at surgery without biopsy of frozen sections, all but one reported patient underwent total nephro-ureterectomy; however, in view of the benign course of the disease (3), we believe that conservative surgery is the treatment of choice. Tony M. Deeths, M.D. Mallinckrodt Institute of Radiology Washington University School of Medicine 510 S. Kingshighway St. Louis, Mo. 63110
DISCUSSION Amyloidosis refers to a variety of conditions associated with tissue infiltration by insoluble proteins and/or protein/polysaccharide complexes. In a small percentage of cases-3 % in one series (2)-amyloid is localized to one tissue or organ; in all of these cases, abnormal collections of plasma cells were found next to the amyloid deposits. We were able to find 10 reports of localized amyloidosis of the ureter, involving 6 women and 4 men ranging from the second to the eighth decade. The lower third of the ureter was involved in 7 cases, whereas the other 3 cases involved the upper third: of these 3, amyloid was found in the renal pelvis in 2. All but one patient had unilateral involvement. Although our patient
REFERENCES 1. Gardner KD Jr, Castellino RA, Kempson R, et al: Primaryamyloidosis of the renal pelvis. N Engl J Moo 284:1196-1198,27 May 1971 2. Isobe T, Osserman ERF: Patterns of amyloidosis and their association with plasma-cell dyscrasia, monoclonal immunoglobulins and Bence-Jones proteins. N Engl J Med 290:473-477, 28 Feb 1974 3. Johnson HW, Ankenman GJ: Bilateral ureteral primary amyloidosis. J Urol 92:275-277, Oct 1964 4. Magri J, Atkinson EA: Primary amyloidosis of the ureter. Br J Urol 42:37-42, Feb 1970
-1-F;~m the Mallinckrodt Institute of Radiology, Washington University School of Medicine, St.Louis, Mo. Accepted for publication in Februa~y 1976.
sjh
60
Localized Amyloidosis of the Ureter 1 King 1ak Lee, M.D., and 10ny M. Deeths, M.D. A case of localized amyloidosis of the distal ureter in a 51-year-old man is described. Pertinent features of previously reported cases are described, and the difficulty of distinguishing this entity from primary carcinoma of the ureter is discussed. INDEX TERMS:
Amyloidosis. Ureters, diseases. Ureters, neoplasms
Radiology 120:60, July 1976
of the urinary tract is relatively ~ncom mon, and localized involvement of the ureter IS even less frequent. Only 10 such cases have been reported (3, 4). We wish to add the following case. AMYLOIDOSIS
.K
CASE REPORT A 51-year-old white man was evaluated following the sudden onset of colicky left flank pain and hematuria. Physical examination revealed mild tenderness of the left costovertebral angle. Urinalysis showed more than 50 red blood cells and 2 white blood cells per high-power field, but no protein or bacteria. Routine laboratory tests were normal. Excretory urography demonstrated delayed function on the left and moderate ureteropyelocaliectasis. A stenotic distal ureteral segment measuring 1 em was seen 3 em above the ureterovesical junction with dilatation distal to it, a picture most consistent with neoplasm, although a benign stricture could not be excluded. At cystoscopy, the bladder and ureteral orifices were normal. Retrograde pyelography (Fig. 1) showed changes similar to those seen on the urogram. Cytological examination of brushings from the stenotic region was negative. At exploratory laparotomy, the constricted area was found to be rock-hard. The high suspicion of carcinoma and the technical difficulty of doing a partial ureteral resection in this case necessitated a left nephro-ureterectomy. Microscopic examination of the indurated area of the ureter revealed amyloid deposits beneath a denuded mucosa, with mild infiltration by lymphocytes and a few multinucleated giant cells. The arteries were surrounded by amyloid deposits. The kidney itself was normal.
Fig. 1. Retrograde pyelogram demonstrating proximal and distal dilatation, irregular shelving at the margins of the stricture, and the small caliber of the strictured region.
had the usual presenting symptoms of flank pain and hematuria, he did not have the insidious onset of symptoms characteristic of other reported cases. Although submucosal calcification is pathognomonic of primary amyloidosis of the renal pelvis and calyces (1), localized amyloid of the ureter has no distinctive appearance and is generally indistinguishable from strictures or from intrinsic or extrinsic neoplasm. Because of the nonspecificity of the radiographic findings, and because tumor is difficult to exclude at surgery without biopsy of frozen sections, all but one reported patient underwent total nephro-ureterectomy; however, in view of the benign course of the disease (3), we believe that conservative surgery is the treatment of choice. Tony M. Deeths, M.D. Mallinckrodt Institute of Radiology Washington University School of Medicine 510 S. Kingshighway St. Louis, Mo. 63110
DISCUSSION Amyloidosis refers to a variety of conditions associated with tissue infiltration by insoluble proteins and/or protein/polysaccharide complexes. In a small percentage of cases-3 % in one series (2)-amyloid is localized to one tissue or organ; in all of these cases, abnormal collections of plasma cells were found next to the amyloid deposits. We were able to find 10 reports of localized amyloidosis of the ureter, involving 6 women and 4 men ranging from the second to the eighth decade. The lower third of the ureter was involved in 7 cases, whereas the other 3 cases involved the upper third: of these 3, amyloid was found in the renal pelvis in 2. All but one patient had unilateral involvement. Although our patient
REFERENCES 1. Gardner KD Jr, Castellino RA, Kempson R, et al: Primaryamyloidosis of the renal pelvis. N Engl J Moo 284:1196-1198,27 May 1971 2. Isobe T, Osserman ERF: Patterns of amyloidosis and their association with plasma-cell dyscrasia, monoclonal immunoglobulins and Bence-Jones proteins. N Engl J Med 290:473-477, 28 Feb 1974 3. Johnson HW, Ankenman GJ: Bilateral ureteral primary amyloidosis. J Urol 92:275-277, Oct 1964 4. Magri J, Atkinson EA: Primary amyloidosis of the ureter. Br J Urol 42:37-42, Feb 1970
-1-F;~m the Mallinckrodt Institute of Radiology, Washington University School of Medicine, St.Louis, Mo. Accepted for publication in Februa~y 1976.
sjh
60
