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Urol Int 1991;47:164-166

Primary Localized Amyloidosis of the Ureter Associated with Osseous Metaplasia Kunio Yamaguchia , Norikazu Kitagawa3, Tadashi Kotake*, Osamu M atsuzakib, Koichi Nagaob, Haruo Ito* Departments o f aUrology and ’’Pathology, Teikyo University School of Medicine, Ichihara Hospital, Ichihara, Japan

Key Words. Amyloidosis, ureter • Osseous metaplasia Abstract. A case of primary localized amyloidosis of the ureter associated with osseous metaplasia, presenting as a calcification on computerized tomography, is reported. We believe this is the second such case to be reported in the world literature. The previous 34 cases of primary amyloidosis of the ureter are reviewed and the clinical presenta­ tions and treatments discussed. The difficulties of distinguishing the condition from transitional cell carcinoma of the ureter is emphasized. Surgical treatment should be conservative, if possible.

Primary amyloidosis of the ureter is a very rare con­ dition. The first case was reported by Lehmann [1] in 1937. I

Since then, only 33 cases have been reported in the world literature, to our knowledge. Of them osseous metaplasia was found in 4 cases. We present a case of amyloidosis of the ureter associated with osseous meta­ plasia of the ureteral wall. 2

Fig. 1. An IVP revealed left hydroureteronephrosis and filling defect of the lower portion of the left ureter. Ureteral calculus was not detected. Fig. 2. Calcification of the left ureteral wall was identified in computerized tomography of the pelvis.

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Introduction

Primary Amyloidosis of Ureter

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Fig. 3. The amyloid tumor of the ureteral wall. A large confluent mass of amyloid in the propria muscle layer and subserosal layer of the ureter. Focally, an osseous metaplasia in the amyloid tumor is seen. HE. X 10. Fig. 4 . A higher magnification of the amyloid tumor. Histologic exami­ nation revealed ossification with bone marrow formation of the left ureter and deposition of homoge­ neous amyloid in the submucosal layer. HE. X 25.

Case Report

Fig. 5. A higher magnification of amyloid tumor of ureter. The perivascular and interstitial amorphous material in the ureteral wall showed positive reaction for Congo der staining. X 50.

submucosal to subadventitial layer, especially the adventitial vessel walls (fig. 3). Characteristically, bone formation with bone marrow and calcification were evident at the site of amyloid deposition (fig. 4). The amyloid showed positive Congo red staining with an intense green birefringence by polarized light. The amyloid tissue did not stain with potassium permanganate, indicating an AA type of amyloid (fig. 5) [2]. The left kidney and proximal portion of the ureter showed no evidence of amyloidosis.

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A 50-year-old woman presented in July 1988 with left flank pain. She had no urological history, other medical problems or previous surgery, and she was not on medication. A sonogram of the abdo­ men revealed left mild hydroureteronephrosis. Except for urinalysis, all other laboratory values were within normal limits. Five to 10 red blood cells per field were identified in the urine sediment. Urine cytology revealed no malignant cells. An excretory urogram (IVP) revealed left hydroureteronephrosis just proximal to the level of spina iliaca anterior superior at which there appeared to be a ureteral stricture or filling defect (fig. 1). No calcification was identified on the plain film. Cystoscopy was nor­ mal. Computerized tomography (CT) of the abdomen revealed cal­ cifications in the area of the ureteral lesion (fig. 2). The patient was followed carefully with no treatment for 1 month. An IVP after 1 month revealed that left hydroureterone­ phrosis was progressive. BCG skin testing was negative. Exploratory laparotomy was performed under general anesthesia to establish the diagnosis. The ureter was thickened, forming a hard stony mass. An approximately 5-cm length of the distal portion of the left ureter was involved by a similar process. Although biopsies from this area were performed, pathological examination of the fro­ zen section could not rule out ureteral malignancy. Left nephroureterectomy and partial cystectomy were performed. The kidney showed dilatation of the renal pelvis and the upper half of the ureter was also dilated by stenotic thickening of the mid­ dle part of the ureter. The stenotic part of the ureter felt stony and hard. On microscopic examination, the middle part of the ureter showed an almost intact urothelium with chronic inflammatory changes and the presence of extensive amyloid deposition in the

To rule out systemic amyloidosis, ultrasonographic examination of the liver and rectal biopsy were performed. To rule out secondary amyloidosis, serum immunoglobulin and RA test were performed. No abnormalities were found on any examinations. We concluded the definite diagnosis of primary organ-limited amyloidosis of the ureter.

Comment Amyloidosis was formerly classified as primary or secondary [3]. Tissue biopsy plays an important role in the diagnosis of primary amyloidosis. Easy accessibility and the absence of serious complications make the rec­ tum the primary site for initial biopsy as in our case. Twenty-two cases of amyloidosis of the ureter were reviewed previously by Gulmi et al. [4]. We added 11 cases and the present case to their report and analyzed 34 cases of primary amyloidosis of the ureter in the litera­ ture [5-15], Analysis of the 34 reported cases of primary amyloid­ osis of the ureter revealed that the age distribution was from 12 to 81 (mean 54) years of age and the male to female ratio is 1:1.8. The majority of patients presented with hematuria or signs and symptoms of ureteral ob­ struction. In 23 of the 34 cases, the diagnosis was not made preoperatively, a ureteral malignancy was suspected and nephroureterectomy was performed as in our case. Frozen section diagnosis in other cases has enabled local excision. To prevent the unnecessary loss of a kid­ ney, persistent effort on obtaining tissue or cytologic evi­ dence of malignancy is a required precondition to neph­ roureterectomy. A flexible and rigid ureteroscopy and biopsy may be extremely helpful in confirming benign ureteral lesions such as amyloidosis. Moul and McLeod [15] reported the only cases in which osseous metaplasia and radiographic calcification were documented. In our case calcification of the ureteral wall was not visualized on plain film, but was on CT scan. The histo­ logical examination revealed osseous metaplasia in the ureteral wall. Higbee and Millett [16] were the only authors to report osseous metaplasia associated with ureteral amy­ loidosis, although they did not document radiographic calcification. Our case is the second to be reported and in which radiographic calcification is documented.

Yamaguchi/Kitagawa/Kotake/Matsuzaki/Nagao/Ito

References 1 Lehmann G: Über örtliche Amyloidablagerung (lokales Amy­ loid) in der Wand des Harnleiters. Zentralbl Allg Pathol 1937; 68:209-213. 2 Wright JR, Calkins E, Humphrey RL: Potassium permanganate reaction in amyloidosis. A histologic method to assist in differ­ entiating forms of this disease. Lab Invest 1977;36:274-281. 3 Glenner GG: Amyloid deposits and amyloidosis. N Engl J Med 1980;302:1283-1342. 4 Gulmi FA, Mooppan UMM, Gomez-Leon G, Kim H: Primary localized amyloidosis of ureter. Urology 1988;32:161-164. 5 Furushima H, Endo M: A case of amyloidosis of the kidney and ureter. Jap J Urol 1972;63:982. 6 Lee KT, Deeths TM: Localized amyloidosis of the ureter. Radi­ ology 1976; 120:60. 7 Okumura H, Imoto T, Matsuura H. Matsumoto K, Yamabe H: Two cases of localized amyloidosis of the urinary tract. Jap J Urol 1979;70:1031. 8 Ogawa H, Ono Y, Kinukawa T, Matsuura O, Takeuchi N, Hirabayashi S, Umeda S, Ohshima S, Shimoji T, Mitsuya H, Hirabayashi N: Localized amyloidosis of the ureter treated by renal autotransplantation: Report of a case. Acta Urol Jpn 1980;26: 1125-1130. 9 Minakata S, Yamada H, Senzaki A: A case of a localized amy­ loidosis of the ureter. Acta Urol Jpn 1982;28:431-437. 10 Husain A, McLay AL, Paterson PJ: Amyloidosis of the ureter. Br J Urol 1984;56:438-439. 11 Mullin EM Jr, Trostle DR, Fetzer AE, Jaffe JS, Suraci A, Shane JJ: Bilateral amyloidosis of the ureter associated with carcino­ ma. J Urol 1984;132:1181-1183. 12 Primrose JN, McKean M, Desai S: Primary amyloidosis of ure­ ter. Urology 1985;25:650-652. 13 Davis PS, Babaria A, March DE, Goldberg RD. Urol Radiol 1987;9:158-160. 14 Loke SL, Ma L, Yiu TF, Wong KK: Localized amyloidosis of the ureter. Pathology 1987;19:91-94. 15 Moul JW, McLeod DG: Bilateral organ-limited amyloidosis of the distal ureter associated with osseous metaplasia and radiographic calcification. J Urol 1988;139:807-809. 16 Higbee DR, Millett WD: Localized amyloidosis of the ureter: Report of a case. J Urol 1956;75:424-427.

Received: July 6, 1990 Accepted: January 29, 1991 Kunio Yamaguchi, MD Department of Urology Teikyo University School of Medicine Ichihara Hospital 3426-3 Anesaki Ichihara-shi 299-01 (Japan)

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Primary localized amyloidosis of the ureter associated with osseous metaplasia.

A case of primary localized amyloidosis of the ureter associated with osseous metaplasia, presenting as a calcification on computerized tomography, is...
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