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Localized Amyloid Tumor of the Ureter: Report of A Case
Vol. 105, April Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1971 by The Williams & Wilkins Co.
LOCALIZED AMYLOID TUMOR OF THE URETER: REPORT OF A CASE MINATO TAKARA, HAJIME NAGATA
AND
TAKAO SONODA
From the Department of Urology, Osaka University Hospital, Osaka, Japan
Localized amyloid tumor of the urinary tract is rare. There have been 49 cases reported and more than half of these cases involved the bladder. Only 8 cases involving the ureter have been documented since 1937 (see table.) 1-s Clinical features of the localized amyloid tumor of the ureter are similar to those of ureteral lithiasis, stricture and neoplasm. CASE REPORT
A 37-year-old man was admitted to the hospital on September 16, 1968 with dull pain in the lower left side of the abdomen. He had had left flank pain and cloudy urine for about 2 years. Physical examination revealed a well-nourished man without previous record of any particular disease. Blood pressure was 130/80 mm. Hg and pulse rate and temperature were normal. Erythrocytes were 482 times 104 per cu. mm., leukocytes were 7,100 per cu. mm. and blood sedimentation rate was 4 mm. per hour. Laboratory findings, including blood chemistry values, liver function tests and serological examinations, were normal. Urinalysis disclosed acidic cloudy urine containing many red and white blood cells without protein. On scout film small calcified shadows were visible in the region of upper third of the left
ureter. Cystoscopic findings were normal. Excretory urography and left retrograde pyelography revealed a normal left caliceal system and dilatation of the ureter down to an irregular narrowing of the upper third portion (fig. 1). Abnormal vascularity was not shown in the region of the left urinary tract on abdominal aortography. With the tentative diagnosis of left ureteral tumor total nephroureterectomy was performed on October 7. During exploration the upper third of the left ureter was dilated. The wall of the ureter in this region was thicker than normal and was hard on palpation. A specimen of the ureter was hard and irregular on its surface and had several small stones consisting of
Accepted for publication April 13, 1970. Lehmann, G.: Ueber iirtliche Amyloidablagerung (lo kales Amyloid) in der Wand des Harnleiters. Zbl. Allg. Path., 68: 209, 1937. 2 Gilbert, L. W. and McDonald, J. R.: Primary amyloidosis of the renal pelvis and ureter: report of case. J. Urol., 68: 137, 1952. 3 Higbee, D. R. and Millett, W. D.: Localized amyloidosis of the ureter: report of a case. J. U rol., 75: 424, 1956. 4 Sato, S.: Primary amyloidosis of the renal pelvis and ureter: report of a case. Acta med. et biol., 5: 15, 1957. 5 Andreas, B. F. and Oosting, M.: Primary amyloidosis of the ureter. J. Urol., 79: 929, 1958. 6 Konrath, M. and Miiebius, G.: Uber tumorformige Paraamyloidose des Ureter. Zbl. Allg. Path., 101: 195, 1960. 7 Johnson, H. W. and Ankenman, G. J.: Bilateral ureteral primary amyloidosis. J. U rol., 92: 275, 1964. 8 Y alowitz, P. A. and Kelalis, P. P.: Primary amyloidosis of the ureter: report of case. J. Urol., 96: 668, 1966. 1
502
Frn. 1. Left retrograde pyelogram reveals dilatation of upper third of left ureter and filling defect at left transverse processus of 4th lumbar vertebra.
LOCALIZED AMYLOID TUMOR OF THE URETER
calcium oxalate which were 8 cm. from the ureteropelvic junction. Hematoxylin and eosin staining demonstrated eosinophilic substances prominent perivascularly in the submucosal layer of the ureter (fig. 2). These homogeneous substances were periodic
503
acid Schiff positive, were stained reddish by Congo red and metachromasia was shown at pH 7.0 in toluidine blue staining (fig. 3). However, there were no pathological changes except for a slight infiltration of small round cells in the renal pelvis.
Fm. 2. Microscopic view shows eosinophilic, homogeneous substance shown perivascularly in submucosal layer of ureter. H & E.
Fm. 3. A, microscopic view with periodic acid Schiff stain. B, toluidine blue stain shows metachromasia is most strongly demonstrated at pH 7.0.
504
TAKARA, NAGATA AND SONODA
Summary of cases of localized amyloid tumor of the ureter Author Lehmannl Gilbert and McDonald' Higbee and Millett' Sato 4 Andreas and Oosting 5 Konrath and MOebius 6 Johnson and Ankenman7
Side
Localization
67-F 52-F
Lt. Lt.
Lower third Upper third
Abdominal pain Lt. flank pain
71-F
Rt.
Lower third
37-M
Rt.
Upper third
Fever, cloudy urine and rt. back pain Gross hematuria
Autopsy Lt. renal lithiasis with lt. ureteral stricture Rt. hydronephrosis and hydrff-ureter Rt. papillomatosis
12-F
Lt.
Lower third
55-M
Lt.
Lower third
Abdominal pain, gross hematuria Lt. colicky pain
Lt. ureteral tumor or lt. tuberculous ureteritis Lt. ureteral stricture
17-M
Bil.
Rt. lower end
Lt. ureteral tumor Lt. ureteral tumor
Main Complaint
73-F
Lt.
Lower third
Rt. flank pain with gross hematuria Lt. flank pain with gross hematuria Lt. flank pain
37-M
Lt.
Upper third
Lt. lower abdominal
Lt. lower end Yalowitz and Kelalis 8 Present case
Preop. Diagnosis
Age-Sex
pain
Convalescence was uneventful and followup data after 14 months were normal. COMMENT
It is well known that amyloidosis is accompanied by primary diseases such as tuberculosis, leprosy, syphilis, multiple myeloma, malignant tumors, etc. In the urogenital tract, amyloid depositions are most frequently found in the renal parenchyma but these are always seen in primary and/or secondary systemic amyloidosis. Localized amyloid tumor belongs to a special category of amyloidosis. 9 • 10 The larynx, trachea and bronchus are frequently involved by this tumor. Symmers stated that the nasal cavity, urogenital organs, skin, cornea and vaginal mucosa are also sites of this lesion. We found 49 case reports of localized amyloid tumors of the urinary tract in the literature. Localization of amyloid depositions in these cases, including our case is: 29 cases in the bladder, 11- 16 9 in the ureter,1-s 9 in the urethra17 9 Symmers, W. St. C.: Primary amyloidosis: a review. J. Clin. Path., 9: 187, 1956. 1 ° Cohen, A. S.: Amyloidosis. New Engl. J. Med., 277: 522, 1967. 11 Kinzel, R. C., Harrison, E. G., Jr. and Utz, D. C.: Primary localized amyloidosis of the bladder. J. Urol., 85: 785, 1961. 12 Werner, H.: Ein primares tumorfiirmiges Amyloid der HarnHase. Z. Urol., 54: 61, 1961. 13 Nagel, R.: Localized amyloidosis of the bladder. J. Urol., 88: 56, 1962. 14 Grace, D. A. and Walton, K. N.: Primary
Operation
Lt. nephrectomy Rt. total nephroureterectomy Rt. total nephroureterectomy Lt. total nephroureterectomy Lt. total nephroureterectomy Rt. ureterocystoneostomy Lt. ureterocystoneostomy Lt. total nephroureterectomy Lt. total nephroureterectomy
and 5 in the renal pelvis. 2• 4 • 18 • 19 In this series, 2 patients had lesions in both the renal pelvis and the ureter. 2 • 4 Diagnosis of this condition has been established only by histopathological examinations of surgically removed specimens because preoperative clinical examination can not be used to differentiate between the conditions of stricture, stone or malignant neoplasm of the ureter. A biopsy of the lesion during operation seems to be a more reasonable therapeutic procedure. SUMMARY
A case of localized amyloid tumor in the ureter was described and 49 cases of localized amyloid depositions in the urinary tract were reviewed. localized amyloidosis of the bladder. J. U rol., 92: 655, 1964. 15 Narwani, K. P. and Lingard, W. F.: Primary localized amyloidosis of the urinary bladder. Canad. Med. Ass. J., 95: 76, 1966. 16 Tripathi, V. N. P. and Desautels, R. E.: Primary amyloidosis of the urogenital system: a study of 16 cases and brief review. J. Urol., 102: 96, 1969. 17 Branson, A. D., Kiser, W. S., Gifford, R. W., Jr. and Tung, K. S. K.: Localized amyloidosis of the urethra: report of a case. J. Urol., 101: 68, 1969. 18 Akimoto, K.: Uber amyloidartige Eiweissniederschlage im Nierenbecken. Beitr. Path. Anat., 78: 239, 1927. 19 Chisholm, G.D., Cooter, N. B. E. and Dawson, J. M.: Primary amyloidosis of the renal pelvis. Brit. Med. J., 1: 736, 1967.
THE JOURNAL OF UROLOGY
Copyright© 1971 by The Williams & Wilkins Co.
LOCALIZED AMYLOID TUMOR OF THE URETER: REPORT OF A CASE MINATO TAKARA, HAJIME NAGATA
AND
TAKAO SONODA
From the Department of Urology, Osaka University Hospital, Osaka, Japan
Localized amyloid tumor of the urinary tract is rare. There have been 49 cases reported and more than half of these cases involved the bladder. Only 8 cases involving the ureter have been documented since 1937 (see table.) 1-s Clinical features of the localized amyloid tumor of the ureter are similar to those of ureteral lithiasis, stricture and neoplasm. CASE REPORT
A 37-year-old man was admitted to the hospital on September 16, 1968 with dull pain in the lower left side of the abdomen. He had had left flank pain and cloudy urine for about 2 years. Physical examination revealed a well-nourished man without previous record of any particular disease. Blood pressure was 130/80 mm. Hg and pulse rate and temperature were normal. Erythrocytes were 482 times 104 per cu. mm., leukocytes were 7,100 per cu. mm. and blood sedimentation rate was 4 mm. per hour. Laboratory findings, including blood chemistry values, liver function tests and serological examinations, were normal. Urinalysis disclosed acidic cloudy urine containing many red and white blood cells without protein. On scout film small calcified shadows were visible in the region of upper third of the left
ureter. Cystoscopic findings were normal. Excretory urography and left retrograde pyelography revealed a normal left caliceal system and dilatation of the ureter down to an irregular narrowing of the upper third portion (fig. 1). Abnormal vascularity was not shown in the region of the left urinary tract on abdominal aortography. With the tentative diagnosis of left ureteral tumor total nephroureterectomy was performed on October 7. During exploration the upper third of the left ureter was dilated. The wall of the ureter in this region was thicker than normal and was hard on palpation. A specimen of the ureter was hard and irregular on its surface and had several small stones consisting of
Accepted for publication April 13, 1970. Lehmann, G.: Ueber iirtliche Amyloidablagerung (lo kales Amyloid) in der Wand des Harnleiters. Zbl. Allg. Path., 68: 209, 1937. 2 Gilbert, L. W. and McDonald, J. R.: Primary amyloidosis of the renal pelvis and ureter: report of case. J. Urol., 68: 137, 1952. 3 Higbee, D. R. and Millett, W. D.: Localized amyloidosis of the ureter: report of a case. J. U rol., 75: 424, 1956. 4 Sato, S.: Primary amyloidosis of the renal pelvis and ureter: report of a case. Acta med. et biol., 5: 15, 1957. 5 Andreas, B. F. and Oosting, M.: Primary amyloidosis of the ureter. J. Urol., 79: 929, 1958. 6 Konrath, M. and Miiebius, G.: Uber tumorformige Paraamyloidose des Ureter. Zbl. Allg. Path., 101: 195, 1960. 7 Johnson, H. W. and Ankenman, G. J.: Bilateral ureteral primary amyloidosis. J. U rol., 92: 275, 1964. 8 Y alowitz, P. A. and Kelalis, P. P.: Primary amyloidosis of the ureter: report of case. J. Urol., 96: 668, 1966. 1
502
Frn. 1. Left retrograde pyelogram reveals dilatation of upper third of left ureter and filling defect at left transverse processus of 4th lumbar vertebra.
LOCALIZED AMYLOID TUMOR OF THE URETER
calcium oxalate which were 8 cm. from the ureteropelvic junction. Hematoxylin and eosin staining demonstrated eosinophilic substances prominent perivascularly in the submucosal layer of the ureter (fig. 2). These homogeneous substances were periodic
503
acid Schiff positive, were stained reddish by Congo red and metachromasia was shown at pH 7.0 in toluidine blue staining (fig. 3). However, there were no pathological changes except for a slight infiltration of small round cells in the renal pelvis.
Fm. 2. Microscopic view shows eosinophilic, homogeneous substance shown perivascularly in submucosal layer of ureter. H & E.
Fm. 3. A, microscopic view with periodic acid Schiff stain. B, toluidine blue stain shows metachromasia is most strongly demonstrated at pH 7.0.
504
TAKARA, NAGATA AND SONODA
Summary of cases of localized amyloid tumor of the ureter Author Lehmannl Gilbert and McDonald' Higbee and Millett' Sato 4 Andreas and Oosting 5 Konrath and MOebius 6 Johnson and Ankenman7
Side
Localization
67-F 52-F
Lt. Lt.
Lower third Upper third
Abdominal pain Lt. flank pain
71-F
Rt.
Lower third
37-M
Rt.
Upper third
Fever, cloudy urine and rt. back pain Gross hematuria
Autopsy Lt. renal lithiasis with lt. ureteral stricture Rt. hydronephrosis and hydrff-ureter Rt. papillomatosis
12-F
Lt.
Lower third
55-M
Lt.
Lower third
Abdominal pain, gross hematuria Lt. colicky pain
Lt. ureteral tumor or lt. tuberculous ureteritis Lt. ureteral stricture
17-M
Bil.
Rt. lower end
Lt. ureteral tumor Lt. ureteral tumor
Main Complaint
73-F
Lt.
Lower third
Rt. flank pain with gross hematuria Lt. flank pain with gross hematuria Lt. flank pain
37-M
Lt.
Upper third
Lt. lower abdominal
Lt. lower end Yalowitz and Kelalis 8 Present case
Preop. Diagnosis
Age-Sex
pain
Convalescence was uneventful and followup data after 14 months were normal. COMMENT
It is well known that amyloidosis is accompanied by primary diseases such as tuberculosis, leprosy, syphilis, multiple myeloma, malignant tumors, etc. In the urogenital tract, amyloid depositions are most frequently found in the renal parenchyma but these are always seen in primary and/or secondary systemic amyloidosis. Localized amyloid tumor belongs to a special category of amyloidosis. 9 • 10 The larynx, trachea and bronchus are frequently involved by this tumor. Symmers stated that the nasal cavity, urogenital organs, skin, cornea and vaginal mucosa are also sites of this lesion. We found 49 case reports of localized amyloid tumors of the urinary tract in the literature. Localization of amyloid depositions in these cases, including our case is: 29 cases in the bladder, 11- 16 9 in the ureter,1-s 9 in the urethra17 9 Symmers, W. St. C.: Primary amyloidosis: a review. J. Clin. Path., 9: 187, 1956. 1 ° Cohen, A. S.: Amyloidosis. New Engl. J. Med., 277: 522, 1967. 11 Kinzel, R. C., Harrison, E. G., Jr. and Utz, D. C.: Primary localized amyloidosis of the bladder. J. Urol., 85: 785, 1961. 12 Werner, H.: Ein primares tumorfiirmiges Amyloid der HarnHase. Z. Urol., 54: 61, 1961. 13 Nagel, R.: Localized amyloidosis of the bladder. J. Urol., 88: 56, 1962. 14 Grace, D. A. and Walton, K. N.: Primary
Operation
Lt. nephrectomy Rt. total nephroureterectomy Rt. total nephroureterectomy Lt. total nephroureterectomy Lt. total nephroureterectomy Rt. ureterocystoneostomy Lt. ureterocystoneostomy Lt. total nephroureterectomy Lt. total nephroureterectomy
and 5 in the renal pelvis. 2• 4 • 18 • 19 In this series, 2 patients had lesions in both the renal pelvis and the ureter. 2 • 4 Diagnosis of this condition has been established only by histopathological examinations of surgically removed specimens because preoperative clinical examination can not be used to differentiate between the conditions of stricture, stone or malignant neoplasm of the ureter. A biopsy of the lesion during operation seems to be a more reasonable therapeutic procedure. SUMMARY
A case of localized amyloid tumor in the ureter was described and 49 cases of localized amyloid depositions in the urinary tract were reviewed. localized amyloidosis of the bladder. J. U rol., 92: 655, 1964. 15 Narwani, K. P. and Lingard, W. F.: Primary localized amyloidosis of the urinary bladder. Canad. Med. Ass. J., 95: 76, 1966. 16 Tripathi, V. N. P. and Desautels, R. E.: Primary amyloidosis of the urogenital system: a study of 16 cases and brief review. J. Urol., 102: 96, 1969. 17 Branson, A. D., Kiser, W. S., Gifford, R. W., Jr. and Tung, K. S. K.: Localized amyloidosis of the urethra: report of a case. J. Urol., 101: 68, 1969. 18 Akimoto, K.: Uber amyloidartige Eiweissniederschlage im Nierenbecken. Beitr. Path. Anat., 78: 239, 1927. 19 Chisholm, G.D., Cooter, N. B. E. and Dawson, J. M.: Primary amyloidosis of the renal pelvis. Brit. Med. J., 1: 736, 1967.