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Primary amyloidosis of renal pelvis and renal cortical adenoma
PRIMARY AMYLOIDOSIS AND RENAL CORTICAL MARTIN GELBARD,
M.D.
STEVEN
M.D.
JOHNSON,
OF RENAL PELVIS ADENOMA
From the Urology Division, Wadsworth Veterans Administration Hospital, and the Department of Surgery, Division of Urology, UCLA School of Medicine, Los Angeles, California
ABSTRACT -A case of primary renal pelvic amyloidosis presenting with spontaneous extrauasation is discussed. The association with an ipsilateral renal cortical adenoma is noted. A review of the pertinent literature is given.
Primary amyloidosis of the renal collecting system is unusual.‘-” Ureteral involvement has been reported with greater frequency than renal pelvic involvement. The following case demonstrates primary amyloidosis of the renal pelvis and proximal ureter, as well as an interesting association with a renal cortical adenoma. Case Report A sixty-seven-year-old accountant was referred for evaluation of left flank pain and gross total hematuria. The pain was described as dull and intermittent. The hematuria accompanied the pain and gradually cleared as the pain resolved over a five-day period. Swelling of the left flank was also reported by the patient, noted following the onset of hematuria. He related that it had resolved a few days later. He reported no voiding symptoms, no prior genitourinary surgery, no history of urolithiasis, and no history of contact or infection with tuberculosis or parasitic disease. Foreign travel included Europe and Mexico. The patient’s past medical history included a cardiovascular accident incurred during a total hip replacement for osteoarthritis, with mild residual depressive reaction. His medications were amitriptyline methylphenidate (Ritalin), and (Elavil), flurazepam (Dalmane). On physical examination mild left flank tenderness was noted. Examination of the genitalia
614
and prostate was unremarkable. Laboratory evaluation revealed sterile urine on culture, 5 to 10 red blood cells per high-powered field, and peripheral eosinophilia with 21 per cent eosinophils in a total white count of 8,800. The serum creatinine was 1.4 mg.1100 ml. and hemoglobin 15.7 Gm./lOO ml., with normal electrolytes, serum 12-panel chemistries, clotting studies, electrocardiogram, and chest roentThere was no proteinuria, and genogram. cutaneous purified protein derivative testing was nonreactive. An intravenous pyelogram (IVP) revealed prompt excretion bilaterally with smooth cortical outlines and a normal right collecting system (Fig. 1). Dilatation and beading of the proximal left ureter was noted, with extravasation of contrast material medially from the region of the left ureteropelvic junction. No hydronephrosis or clubbing was seen. On cystoscopic examination, moderate trilobar prostatic enlargement was noted with a trabeculated, but otherwise normal-appearing bladder. Cytologic studies obtained from the left system by catheterization and barbotage were negative. A left retrograde pyelogram confirmed the IVP findings, with extravasation observed at small volume and low pressure filling (Fig. 2). The distal left ureter appeared normal on drainage film. At this point the patient was admitted for angiography. This demonstrated a 5 by 5-cm. avascular mass in the lower pole of the left kidney
UROLOGY
/
JUNE 1960
/
VOLUME
XV, NUMBER6
FIGURE 1.
FIGURE 2.
Intravenous pyelograms, (A) jive-minute
L& retrograde pyelogram.
with an otherwise normal vascular pattern; continued extravasation was noted in the pyelogram phase. The mass was subsequently shown to be a simple cyst by ultrasound, puncture, and cyst fluid cytology. Exploration was then undertaken to ascertain the nature of the spontaneous left ureteropelvic junction extravasation in the face
UROLOGY
/ JUNE1980 / VOLUMEXV,NUMBER6
film and (B) tomogram.
of a normal angiogram and negative left ureteropelvic cytologic studies. Through a supratwelfth rib left flank incision, the renal vessels were secured, the wound packed, and Gerota fascia opened. Peripelvic fibrosis was encountered and a biopsy taken. Frozen section examination disclosed desmoplasia with atypical foamy histiocytes, as well as a giant cell inflammatory response, but no evidence of neoplasia. Biopsy of a nodular cortical irregularity was taken and found on frozen section to be a welldifferentiated neoplasm, probably a cortical adenoma. Owing to this histologic uncertainty, a left radical nephrectomy was performed. Postoperative recovery was uneventful. Pathologic examination of the specimen confirmed the cortical lesion to be an adenoma (Fig. 3A), with the remainder of the renal parenchyma normal histologically, except for minimal hyaline arteriolosclerosis. The simple cyst was also confirmed. Extensive amyloid deposits, demonstrated by green birefringence of Congo-red staining under polarized light, were seen throughout the wall of the renal pelvis (Fig. 3B). Sections of the proximal ureter also showed widespread transmural amyloidosis. Amyloid deposits as well as extensive histiocytic foreign body reaction with fat necrosis and hemosiderin deposition were detected in the fibroadipose peripelvic tissues. In view of the serum and urine immunoelecamyloidosis, trophoresis was obtained and found to be normal. Congo-red staining with polarized light microscopy failed to demonstrate amyloid in the renal parenchyma or in the periarteriolar hyaline material.
615
FIGURE 3. (A) Renal cortical adenoma (hematoxylin and eosin, original magnijkation vie amyloid (Congo-red with polarization, original mugnijkation x 30).
Comment Amyloidosis has been subject to several classification schemes. In a review of 236 cases seen at the Mayo Clinic from 1960 to 1972, Kyle and Bayrdl used the fbllowing. 1. Primary amyloidosis, generalized 2. Amyloidosis with multiple meloma 3. Secondary amyloidosis (associated with rheumatic disease or chronic infections) Localized (single organ) amyloidosis Z: Familial amyloidosis Our case falls into group 4 which comprised only 9 per cent of all cases in their series. With the exception of localized amyloid in the seminal vesicles, believed by some to be a senile degenerative process’ (up to 9 per cent autopsy incidence2), localized disease in the urogenital tract is rare. Lesions of the bladder are the most common with about 30 cases reporteda Thirteen cases of primary ureteral amyloidosis have involvement been reported, 4 with transmural the rule. In 1967 Chisholm, Cooter, and Dawson5 reported 2 cases of primary renal pelvic amyloidosis. One case presented with azotemia secondary to partial obstruction and acute
616
X 75). (B) Renal pel:
pyelonephritis. The second presented with urinary tract infection and renal pelvic calcification indistinguishable from staghorn calculus. In addition, they reviewed 3 previous cases, 2 presenting with hydronephrosis and 1 with irregularity of the upper ureter and pelvis on retrograde pyelography. Since then 2 additional cases have been reported. The first presented with flank pain, microhematuria, and distortion of the pyelocalyceal systems bilaterally on IVP, as well as characteristic thin pyelocalyceal calcifications.g The second, most recent case, was reported in this journal in October, 1979.l’ It appears that our case, the eighth reported, is the first to present with spontaneous extravasation from the upper urinary tract. The patient’s medical history and laboratory evaluation gave no reason to suspect secondary amyloidosis. Of interest, however, was the giant cell reaction observed and said to be less common in primary amyloidosis.’ The absence of any parenchymal deposition in the kidney is strong evidence against disseminated multiple organ involvement. Indeed, it is more conclusive than the results of rectal biopsy which are positive in 84 per cent of patients with systemic disease. Renal biopsy is positive in 90 per cent,
UROLOGY
! JUNE1980 I VOLUMEXV,NUMBER6
second in sensitivity only to the liver biopsy which was positive in 94 per cent.’ Nonetheless, on initial presentation of nonsurgical cases, the rectal biopsy remains the safest source of tissue and should be obtained first. Another apparently unique feature of this case is its association with an ipsilateral renal cortical adenoma. This association has not been described previously, in contrast to the one that exists between renal cell carcinoma and amyloidosis. Coexistent renal cell carcinoma and amyloidosis have been reported in 50 cases.’ In a series of 50 autopsies done on patients with renal cell carcinoma, Penman and Thompson’ noted widespread amyloidosis in 1 case and limited amyloidosis in 2 others. UCLA School of Medicine Center for the Health Sciences (66-143) Los Angeles, California 90024 (DR.
UROLOGY
/
JUNE
1986
/
VOLUME
GELBARD)
XV, NUMBER
6
References 1. Kyle R, and Bayrd E: Amyloidosis: review of 236 cases, Medicine 54: 271 (1975). 2. Goldman H: Amyloidosis of seminal vesicles and vas deferem, Arch. Pathol. 75: 94 (1963). 3. Tripathi V, and De Sautels R: Primary amyloidosis of the urogenital system: a study of 16 cases and brief review, J. Ural. 10.2: 96 (1969). 4. Mariani A, Barrett D, Kurtz S, and Kyle R: Bilateral localized amyloidosis of the ureter presenting with anuria, ibid. 120: 757 (1978). 5. Chisholm G, Cooter N, and Dawson J: Primary amyloidosis of the renal pelvis, Br. Med. J. 1: 736 (1967). 6. Gardener KD, et al: Primary amyloidosis of the renal pelvis, N. Engl. J. Med. 284: 1196 (1971). 7. Penman H, and Thompson K: Amyloidosis and renal cell adenocarcinoma: a postmortem study, J. Pathol. 107: 45 (1972). 8. Johnson H, and Ankeman G: Bilateral ureteral primary amyloidosis, J. Urol. 92: 275 (1964). 9. Magri J, and Atkinson M: Primary amyloidosis of the ureter, Br. J. Urol. 42: 37 (1970). 10. Thomas S, Sanders P, and Pollack H: Primary amyloidosis of urinary bladder and ureter, Urology 9: 586 (1977). 11. Dias R, et al: Amyloidosis of renal pelvis and urinary bladder, ibid. 14: 491 (1979).
617
M.D.
STEVEN
M.D.
JOHNSON,
OF RENAL PELVIS ADENOMA
From the Urology Division, Wadsworth Veterans Administration Hospital, and the Department of Surgery, Division of Urology, UCLA School of Medicine, Los Angeles, California
ABSTRACT -A case of primary renal pelvic amyloidosis presenting with spontaneous extrauasation is discussed. The association with an ipsilateral renal cortical adenoma is noted. A review of the pertinent literature is given.
Primary amyloidosis of the renal collecting system is unusual.‘-” Ureteral involvement has been reported with greater frequency than renal pelvic involvement. The following case demonstrates primary amyloidosis of the renal pelvis and proximal ureter, as well as an interesting association with a renal cortical adenoma. Case Report A sixty-seven-year-old accountant was referred for evaluation of left flank pain and gross total hematuria. The pain was described as dull and intermittent. The hematuria accompanied the pain and gradually cleared as the pain resolved over a five-day period. Swelling of the left flank was also reported by the patient, noted following the onset of hematuria. He related that it had resolved a few days later. He reported no voiding symptoms, no prior genitourinary surgery, no history of urolithiasis, and no history of contact or infection with tuberculosis or parasitic disease. Foreign travel included Europe and Mexico. The patient’s past medical history included a cardiovascular accident incurred during a total hip replacement for osteoarthritis, with mild residual depressive reaction. His medications were amitriptyline methylphenidate (Ritalin), and (Elavil), flurazepam (Dalmane). On physical examination mild left flank tenderness was noted. Examination of the genitalia
614
and prostate was unremarkable. Laboratory evaluation revealed sterile urine on culture, 5 to 10 red blood cells per high-powered field, and peripheral eosinophilia with 21 per cent eosinophils in a total white count of 8,800. The serum creatinine was 1.4 mg.1100 ml. and hemoglobin 15.7 Gm./lOO ml., with normal electrolytes, serum 12-panel chemistries, clotting studies, electrocardiogram, and chest roentThere was no proteinuria, and genogram. cutaneous purified protein derivative testing was nonreactive. An intravenous pyelogram (IVP) revealed prompt excretion bilaterally with smooth cortical outlines and a normal right collecting system (Fig. 1). Dilatation and beading of the proximal left ureter was noted, with extravasation of contrast material medially from the region of the left ureteropelvic junction. No hydronephrosis or clubbing was seen. On cystoscopic examination, moderate trilobar prostatic enlargement was noted with a trabeculated, but otherwise normal-appearing bladder. Cytologic studies obtained from the left system by catheterization and barbotage were negative. A left retrograde pyelogram confirmed the IVP findings, with extravasation observed at small volume and low pressure filling (Fig. 2). The distal left ureter appeared normal on drainage film. At this point the patient was admitted for angiography. This demonstrated a 5 by 5-cm. avascular mass in the lower pole of the left kidney
UROLOGY
/
JUNE 1960
/
VOLUME
XV, NUMBER6
FIGURE 1.
FIGURE 2.
Intravenous pyelograms, (A) jive-minute
L& retrograde pyelogram.
with an otherwise normal vascular pattern; continued extravasation was noted in the pyelogram phase. The mass was subsequently shown to be a simple cyst by ultrasound, puncture, and cyst fluid cytology. Exploration was then undertaken to ascertain the nature of the spontaneous left ureteropelvic junction extravasation in the face
UROLOGY
/ JUNE1980 / VOLUMEXV,NUMBER6
film and (B) tomogram.
of a normal angiogram and negative left ureteropelvic cytologic studies. Through a supratwelfth rib left flank incision, the renal vessels were secured, the wound packed, and Gerota fascia opened. Peripelvic fibrosis was encountered and a biopsy taken. Frozen section examination disclosed desmoplasia with atypical foamy histiocytes, as well as a giant cell inflammatory response, but no evidence of neoplasia. Biopsy of a nodular cortical irregularity was taken and found on frozen section to be a welldifferentiated neoplasm, probably a cortical adenoma. Owing to this histologic uncertainty, a left radical nephrectomy was performed. Postoperative recovery was uneventful. Pathologic examination of the specimen confirmed the cortical lesion to be an adenoma (Fig. 3A), with the remainder of the renal parenchyma normal histologically, except for minimal hyaline arteriolosclerosis. The simple cyst was also confirmed. Extensive amyloid deposits, demonstrated by green birefringence of Congo-red staining under polarized light, were seen throughout the wall of the renal pelvis (Fig. 3B). Sections of the proximal ureter also showed widespread transmural amyloidosis. Amyloid deposits as well as extensive histiocytic foreign body reaction with fat necrosis and hemosiderin deposition were detected in the fibroadipose peripelvic tissues. In view of the serum and urine immunoelecamyloidosis, trophoresis was obtained and found to be normal. Congo-red staining with polarized light microscopy failed to demonstrate amyloid in the renal parenchyma or in the periarteriolar hyaline material.
615
FIGURE 3. (A) Renal cortical adenoma (hematoxylin and eosin, original magnijkation vie amyloid (Congo-red with polarization, original mugnijkation x 30).
Comment Amyloidosis has been subject to several classification schemes. In a review of 236 cases seen at the Mayo Clinic from 1960 to 1972, Kyle and Bayrdl used the fbllowing. 1. Primary amyloidosis, generalized 2. Amyloidosis with multiple meloma 3. Secondary amyloidosis (associated with rheumatic disease or chronic infections) Localized (single organ) amyloidosis Z: Familial amyloidosis Our case falls into group 4 which comprised only 9 per cent of all cases in their series. With the exception of localized amyloid in the seminal vesicles, believed by some to be a senile degenerative process’ (up to 9 per cent autopsy incidence2), localized disease in the urogenital tract is rare. Lesions of the bladder are the most common with about 30 cases reporteda Thirteen cases of primary ureteral amyloidosis have involvement been reported, 4 with transmural the rule. In 1967 Chisholm, Cooter, and Dawson5 reported 2 cases of primary renal pelvic amyloidosis. One case presented with azotemia secondary to partial obstruction and acute
616
X 75). (B) Renal pel:
pyelonephritis. The second presented with urinary tract infection and renal pelvic calcification indistinguishable from staghorn calculus. In addition, they reviewed 3 previous cases, 2 presenting with hydronephrosis and 1 with irregularity of the upper ureter and pelvis on retrograde pyelography. Since then 2 additional cases have been reported. The first presented with flank pain, microhematuria, and distortion of the pyelocalyceal systems bilaterally on IVP, as well as characteristic thin pyelocalyceal calcifications.g The second, most recent case, was reported in this journal in October, 1979.l’ It appears that our case, the eighth reported, is the first to present with spontaneous extravasation from the upper urinary tract. The patient’s medical history and laboratory evaluation gave no reason to suspect secondary amyloidosis. Of interest, however, was the giant cell reaction observed and said to be less common in primary amyloidosis.’ The absence of any parenchymal deposition in the kidney is strong evidence against disseminated multiple organ involvement. Indeed, it is more conclusive than the results of rectal biopsy which are positive in 84 per cent of patients with systemic disease. Renal biopsy is positive in 90 per cent,
UROLOGY
! JUNE1980 I VOLUMEXV,NUMBER6
second in sensitivity only to the liver biopsy which was positive in 94 per cent.’ Nonetheless, on initial presentation of nonsurgical cases, the rectal biopsy remains the safest source of tissue and should be obtained first. Another apparently unique feature of this case is its association with an ipsilateral renal cortical adenoma. This association has not been described previously, in contrast to the one that exists between renal cell carcinoma and amyloidosis. Coexistent renal cell carcinoma and amyloidosis have been reported in 50 cases.’ In a series of 50 autopsies done on patients with renal cell carcinoma, Penman and Thompson’ noted widespread amyloidosis in 1 case and limited amyloidosis in 2 others. UCLA School of Medicine Center for the Health Sciences (66-143) Los Angeles, California 90024 (DR.
UROLOGY
/
JUNE
1986
/
VOLUME
GELBARD)
XV, NUMBER
6
References 1. Kyle R, and Bayrd E: Amyloidosis: review of 236 cases, Medicine 54: 271 (1975). 2. Goldman H: Amyloidosis of seminal vesicles and vas deferem, Arch. Pathol. 75: 94 (1963). 3. Tripathi V, and De Sautels R: Primary amyloidosis of the urogenital system: a study of 16 cases and brief review, J. Ural. 10.2: 96 (1969). 4. Mariani A, Barrett D, Kurtz S, and Kyle R: Bilateral localized amyloidosis of the ureter presenting with anuria, ibid. 120: 757 (1978). 5. Chisholm G, Cooter N, and Dawson J: Primary amyloidosis of the renal pelvis, Br. Med. J. 1: 736 (1967). 6. Gardener KD, et al: Primary amyloidosis of the renal pelvis, N. Engl. J. Med. 284: 1196 (1971). 7. Penman H, and Thompson K: Amyloidosis and renal cell adenocarcinoma: a postmortem study, J. Pathol. 107: 45 (1972). 8. Johnson H, and Ankeman G: Bilateral ureteral primary amyloidosis, J. Urol. 92: 275 (1964). 9. Magri J, and Atkinson M: Primary amyloidosis of the ureter, Br. J. Urol. 42: 37 (1970). 10. Thomas S, Sanders P, and Pollack H: Primary amyloidosis of urinary bladder and ureter, Urology 9: 586 (1977). 11. Dias R, et al: Amyloidosis of renal pelvis and urinary bladder, ibid. 14: 491 (1979).
617