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Malacoplakia of the Urinary Tract with Spread to the Abdominal Wall
Vol. 107, June Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright © 1972 by The Williams & Wilkins Co.
MALACOPLAKIA OF THE URINARY TRACT WITH SPREAD TO THE ABDOMINAL WALL DANIEL R. SCULLIN
AND
ROBYN HARDY
From the Department of Radiology, Louisiana State University School of Medicine, New Orleans, Louisiana Malacoplakia is characterized by broad, sessile, polypoid plaques which most frequently involve the mucosa of the bladder. 1- 4 This lesion has also been described in the ureter, kidney, testicle, prostate, colon, stomach and retroperitoneum. 1 • 2 • 4- 9 The
etiology is obscure but the disease is associated with Escherichia coli infection in a high percentage of cases. 3 • 4 Pathologically, there is a chronic inflammatory reaction containing the Michaelis-Gutmann body 3 or calcospherite.
Fm. 1. A, selective renal angiogram demonstrates angiographic pattern of hydronephrosis in upper pole. Arrows d~marca te the lateral borders of vascular, mass lesion which has extended through the renal capsule (broken lmes). B, delayed film following angiography shows dilated caliceal system containing laminated calcification (upper arrow). Amorphous calcification (middle arrow) was within renal mass and dilated, tortuous ureter was completely obstructed by malacoplakia (lower arrow). Accepted for publication August 6, 1971. 1 Gibson, T. E., Bareta, J. and Lake, G. C.: Malacoplakia: report of a case involving the bladder and one kidney and ureter. Urol. Int., 1: 5, 1955. 2 Lewis, J. A., Vieralves, G., Landes, R. R. and Powell, L. W.: Malakoplakia of the renal pelvis, calyces and upper ureter: case report. J. Urol., 85: 243, 1961. 3 Melicow, M. M.: Malacoplakia: report of case, review of literature. J. Urol., 78: 33, 1957. 4 Miller, 0. S. and Finck, F. M.: Malacoplakia of the kidney: the great impersonator. J. Urol., 103: 712, 1970. 5 Brown, R. C. and Smith, B. H.: Malacoplakia of the testis. Amer. J. Clin. Path., 47: 135, 1967. 6 Goldman, R. L.: A case of malacoplakia with involvement of the prostate gland. J. Urol., 93: 407, 1965. 7 Finlay-Jones, L. R., Blackwell, J.B. and Papadimitriou, J.M.: Malakoplakia of the colon. Amer. J. Clin. Path., 50: 320, 1968.
CASE REPORT
A 55-year-old black woman was referred to the Earl K. Long Memorial Hospital with right flank pain and a "lump in her back" 4 to 5 weeks in duration. The patient complained of fatigue, loss of appetite and a recent 15-pound weight loss. The patient was cachectic and physical examination disclosed a large, firm mass in the right flank. 8 Terner, J. Y. and Lattes, R.: Malakoplakia of colon and retroperitoneum. Report of a case with a histochemical study of the Michaelis-Gutmann inclusion bodies. Amer. J. Clin. Path., 44: 20, 1965. 9 Yunis, E. J., Estevez, J.M., Pinzon, G. J. and Moran, T. J.: Malacoplakia. Discussion of pathogenesis and report of three cases including one of fatal gastric and colonic involvement. Arch. Path., 83: 180, 1967.
908
MALACOPLAKIA OF URINARY TRACT
Urinalysis revealed 4 to 6 white blood cells per high power field and bacilluria. Urine culture disclosed E. coli. Plain films of the abdomen demonstrated a laminated calcification high in the right upper quadrant with a collection of amorphous calcium several centimeters below. An excretory urogram (IVP) revealed prompt function on the left side with a normal left collecting system. There was nonfunction on the right side up to 20 minutes.
FIG. 2. Lobulated, encapsulated, 5 by 8 by 2 cm. mass occupies middle portion and lower pole of right kidney.
909
Selective catheterization of the right renal artery confirmed that it was displaced superiorly and decreased in caliber and that hydronephrosis was present. In addition, there was a mass lesion arising from the lateral aspect of the mid portion and lower pole of the kidney. This mass was associated with a network of tortuous, bizarre vessels (fig. 1, A), which confirmed that it had extended outside of the expected confines of the renal capsule (fig. 1, A). As the dilated collecting system opacified, the precise location of the intrarenal calcifications and complete obstruction of the ureter were demonstrated (fig. 1, B). At this point the diagnostic impressions were 1) carcinoma of the right kidney, partially necrotic and containing small foci of amorphous calcium; 2) nephrolithiasis and 3) ureteral obstruction, etiology undetermined. Surgical firulings and pathology. At operation the distal ureter was found to be obstructed by a broad, sessile plaque which was firm in consistency and yellowish-brown in color. The ureter contained smaller but otherwise identical plaques. There was a mass lesion arising from the mid portion and lower pole of the right kidney which measured 5 by 8 by 2 cm. This mass was smooth, lobulated and grayishtan in color (fig. 2). A right nephrectomy and ureterectomy were performed. Repeated microscopic study of the renal and ureteral lesions has confirmed the diagnosis of malacoplakia. Subsequent course. Following establishment of the diagnosis in the kidney and ureter,. the patient underwent cystoscopy. Several lesions with the typical gross and microscopic appearance of malacoplakia were found in the bladder. Approximately 8 months postoperatively the patient was found to have several firm, elevated, papular, nodular, erythematous lesions arising from the right anterior abdominal wall and flank (fig. 3). Their surfaces were friable and granular. The adjacent skin was intact but elevated and indurated. These lesions were biopsied and were confirmed as malacoplakia. The diagnosis
FIG. 3. Photograph of abdominal wall demonstrates skin lesions
910
SCULLIN AND HARDY
was confirmed by Dr. Bruce Smith at the Armed Forces Institute of Pathology. The patient's urinary tract infection has been treated with gantrisin. In addition, she received a course of methotrexate in an attempt to control this fulminating disease. Despite this, the lesions have continued to extend and have spread to involve the left abdominal wall. DISCUSSION
The most intriguing aspect of this case is the manner in which the lesion has mimicked renal malignancy clinically and roentgenographically. The combined findings in an elderly woman of weight loss, a palpable flank mass containing radiographically demonstrable amorphous calcium, neovascularity and extension of the mass through the renal capsule at angiography, are usually ample evidence of malignancy. Other non-neoplastic processes which have been reported as presenting with renal enlargement and neovascularity are tuberculosis and xanthogranulomatous pyelonephritis. 10- 12 Tuberculosis was not a serious consideration in this case but the tumefactive form of xanthogranulomatous pyelonephritis probably should have been considered. However, the degree of abnormal vascularity seen in our case is rarely if ever seen in focal xanthogranulomatous in10 Rossi, P., Myers, D. H., Furey, R. and BonfilsRoberts, E. A.: Angiography in bilateral xanthogranulomatous pyelonephritis. Case report. Radiology, 90: 320, 1968. 11 Avnet, N. L., Roberts, T. W. and Goldberg, H. R.: Tumefactive xanthogranulomatous pyelonephritis. Amer. J. Roentgen., 90: 89, 1963. 12 Clark, R. L., McAllister, H. A. and Harrell, J. E.: Case of the month from the AFIP: an exercise in radiologic-pathologic correlation. Radiology, 92: 597, 1969.
volvement of the kidney. The subsequent spread of the disease process to the abdominal wall further suggested a malignant process but re-evaluation of the pathological material from the kidney, ureter, bladder and abdominal wall has confirmed the diagnosis of malacoplakia. Malacoplakia must be added to the widening differential diagnosis of mass lesions of the kidney which demonstrate neovascularity angiographically. The only previous report of an angiogram obtained in a case of malacoplakia was described as compatible with obstructive pyohydronephrosis and apparently there was no evidence of new vessel formation.4 However, the presence of newly formed capillaries, thrombosis and a generally rich vascularity has been noted previously in malacoplakia of the ureter and testis. 1 • 2 • 5 Consequently, Brown and Smith have suggested that vascular lesions may play a role in the etiology of malacoplakia. 5 In the present case, the Michaelis-Gutmann bodies were most prevalent in the lateral margin of the lower pole of the renal lesion. This is also the region in which the abnormal vascularity predominated (fig. 1, A). The significance of this combination of findings and what role, if any, that the rich vascularity played in the genesis or spread of the process remain obscure but of interest in view of Brown and Smith's hypothesis. 5 SUMMARY
A case of malacoplakia involving the kidney, ureter and bladder is presented. Angiographically, the renal lesion resembled carcinoma. Subsequently, the disease process spread to the skin of the abdominal wall. Drs. James Freeman and Jack Holden provided assistance for this study.
THE JOURNAL OF UROLOGY
Copyright © 1972 by The Williams & Wilkins Co.
MALACOPLAKIA OF THE URINARY TRACT WITH SPREAD TO THE ABDOMINAL WALL DANIEL R. SCULLIN
AND
ROBYN HARDY
From the Department of Radiology, Louisiana State University School of Medicine, New Orleans, Louisiana Malacoplakia is characterized by broad, sessile, polypoid plaques which most frequently involve the mucosa of the bladder. 1- 4 This lesion has also been described in the ureter, kidney, testicle, prostate, colon, stomach and retroperitoneum. 1 • 2 • 4- 9 The
etiology is obscure but the disease is associated with Escherichia coli infection in a high percentage of cases. 3 • 4 Pathologically, there is a chronic inflammatory reaction containing the Michaelis-Gutmann body 3 or calcospherite.
Fm. 1. A, selective renal angiogram demonstrates angiographic pattern of hydronephrosis in upper pole. Arrows d~marca te the lateral borders of vascular, mass lesion which has extended through the renal capsule (broken lmes). B, delayed film following angiography shows dilated caliceal system containing laminated calcification (upper arrow). Amorphous calcification (middle arrow) was within renal mass and dilated, tortuous ureter was completely obstructed by malacoplakia (lower arrow). Accepted for publication August 6, 1971. 1 Gibson, T. E., Bareta, J. and Lake, G. C.: Malacoplakia: report of a case involving the bladder and one kidney and ureter. Urol. Int., 1: 5, 1955. 2 Lewis, J. A., Vieralves, G., Landes, R. R. and Powell, L. W.: Malakoplakia of the renal pelvis, calyces and upper ureter: case report. J. Urol., 85: 243, 1961. 3 Melicow, M. M.: Malacoplakia: report of case, review of literature. J. Urol., 78: 33, 1957. 4 Miller, 0. S. and Finck, F. M.: Malacoplakia of the kidney: the great impersonator. J. Urol., 103: 712, 1970. 5 Brown, R. C. and Smith, B. H.: Malacoplakia of the testis. Amer. J. Clin. Path., 47: 135, 1967. 6 Goldman, R. L.: A case of malacoplakia with involvement of the prostate gland. J. Urol., 93: 407, 1965. 7 Finlay-Jones, L. R., Blackwell, J.B. and Papadimitriou, J.M.: Malakoplakia of the colon. Amer. J. Clin. Path., 50: 320, 1968.
CASE REPORT
A 55-year-old black woman was referred to the Earl K. Long Memorial Hospital with right flank pain and a "lump in her back" 4 to 5 weeks in duration. The patient complained of fatigue, loss of appetite and a recent 15-pound weight loss. The patient was cachectic and physical examination disclosed a large, firm mass in the right flank. 8 Terner, J. Y. and Lattes, R.: Malakoplakia of colon and retroperitoneum. Report of a case with a histochemical study of the Michaelis-Gutmann inclusion bodies. Amer. J. Clin. Path., 44: 20, 1965. 9 Yunis, E. J., Estevez, J.M., Pinzon, G. J. and Moran, T. J.: Malacoplakia. Discussion of pathogenesis and report of three cases including one of fatal gastric and colonic involvement. Arch. Path., 83: 180, 1967.
908
MALACOPLAKIA OF URINARY TRACT
Urinalysis revealed 4 to 6 white blood cells per high power field and bacilluria. Urine culture disclosed E. coli. Plain films of the abdomen demonstrated a laminated calcification high in the right upper quadrant with a collection of amorphous calcium several centimeters below. An excretory urogram (IVP) revealed prompt function on the left side with a normal left collecting system. There was nonfunction on the right side up to 20 minutes.
FIG. 2. Lobulated, encapsulated, 5 by 8 by 2 cm. mass occupies middle portion and lower pole of right kidney.
909
Selective catheterization of the right renal artery confirmed that it was displaced superiorly and decreased in caliber and that hydronephrosis was present. In addition, there was a mass lesion arising from the lateral aspect of the mid portion and lower pole of the kidney. This mass was associated with a network of tortuous, bizarre vessels (fig. 1, A), which confirmed that it had extended outside of the expected confines of the renal capsule (fig. 1, A). As the dilated collecting system opacified, the precise location of the intrarenal calcifications and complete obstruction of the ureter were demonstrated (fig. 1, B). At this point the diagnostic impressions were 1) carcinoma of the right kidney, partially necrotic and containing small foci of amorphous calcium; 2) nephrolithiasis and 3) ureteral obstruction, etiology undetermined. Surgical firulings and pathology. At operation the distal ureter was found to be obstructed by a broad, sessile plaque which was firm in consistency and yellowish-brown in color. The ureter contained smaller but otherwise identical plaques. There was a mass lesion arising from the mid portion and lower pole of the right kidney which measured 5 by 8 by 2 cm. This mass was smooth, lobulated and grayishtan in color (fig. 2). A right nephrectomy and ureterectomy were performed. Repeated microscopic study of the renal and ureteral lesions has confirmed the diagnosis of malacoplakia. Subsequent course. Following establishment of the diagnosis in the kidney and ureter,. the patient underwent cystoscopy. Several lesions with the typical gross and microscopic appearance of malacoplakia were found in the bladder. Approximately 8 months postoperatively the patient was found to have several firm, elevated, papular, nodular, erythematous lesions arising from the right anterior abdominal wall and flank (fig. 3). Their surfaces were friable and granular. The adjacent skin was intact but elevated and indurated. These lesions were biopsied and were confirmed as malacoplakia. The diagnosis
FIG. 3. Photograph of abdominal wall demonstrates skin lesions
910
SCULLIN AND HARDY
was confirmed by Dr. Bruce Smith at the Armed Forces Institute of Pathology. The patient's urinary tract infection has been treated with gantrisin. In addition, she received a course of methotrexate in an attempt to control this fulminating disease. Despite this, the lesions have continued to extend and have spread to involve the left abdominal wall. DISCUSSION
The most intriguing aspect of this case is the manner in which the lesion has mimicked renal malignancy clinically and roentgenographically. The combined findings in an elderly woman of weight loss, a palpable flank mass containing radiographically demonstrable amorphous calcium, neovascularity and extension of the mass through the renal capsule at angiography, are usually ample evidence of malignancy. Other non-neoplastic processes which have been reported as presenting with renal enlargement and neovascularity are tuberculosis and xanthogranulomatous pyelonephritis. 10- 12 Tuberculosis was not a serious consideration in this case but the tumefactive form of xanthogranulomatous pyelonephritis probably should have been considered. However, the degree of abnormal vascularity seen in our case is rarely if ever seen in focal xanthogranulomatous in10 Rossi, P., Myers, D. H., Furey, R. and BonfilsRoberts, E. A.: Angiography in bilateral xanthogranulomatous pyelonephritis. Case report. Radiology, 90: 320, 1968. 11 Avnet, N. L., Roberts, T. W. and Goldberg, H. R.: Tumefactive xanthogranulomatous pyelonephritis. Amer. J. Roentgen., 90: 89, 1963. 12 Clark, R. L., McAllister, H. A. and Harrell, J. E.: Case of the month from the AFIP: an exercise in radiologic-pathologic correlation. Radiology, 92: 597, 1969.
volvement of the kidney. The subsequent spread of the disease process to the abdominal wall further suggested a malignant process but re-evaluation of the pathological material from the kidney, ureter, bladder and abdominal wall has confirmed the diagnosis of malacoplakia. Malacoplakia must be added to the widening differential diagnosis of mass lesions of the kidney which demonstrate neovascularity angiographically. The only previous report of an angiogram obtained in a case of malacoplakia was described as compatible with obstructive pyohydronephrosis and apparently there was no evidence of new vessel formation.4 However, the presence of newly formed capillaries, thrombosis and a generally rich vascularity has been noted previously in malacoplakia of the ureter and testis. 1 • 2 • 5 Consequently, Brown and Smith have suggested that vascular lesions may play a role in the etiology of malacoplakia. 5 In the present case, the Michaelis-Gutmann bodies were most prevalent in the lateral margin of the lower pole of the renal lesion. This is also the region in which the abnormal vascularity predominated (fig. 1, A). The significance of this combination of findings and what role, if any, that the rich vascularity played in the genesis or spread of the process remain obscure but of interest in view of Brown and Smith's hypothesis. 5 SUMMARY
A case of malacoplakia involving the kidney, ureter and bladder is presented. Angiographically, the renal lesion resembled carcinoma. Subsequently, the disease process spread to the skin of the abdominal wall. Drs. James Freeman and Jack Holden provided assistance for this study.