- Email: [email protected]
Xanthogranulomatous pyelonephritis unusual clinical presentations
XANTHOGRANULOMATOUS Unusual
Clinical
J. H. MERING, G. W. KAPLAN,
PYELONEPHRITIS
Presentations
M.D. M.D.
A. P. MCLAUGHLIN,
III,
M.D.
From the Division of Urology, University Hospital of San Diego County, University of California, San Diego, California
ABSTRACTThe association of xanthogranulomatous pyelonephritis with renal-cell carcinoma and the loss of a kidney homograft because of this disorder suggest that in certain cases altered immunologic competence might be an etiologic factor in this disease. This entity may develop acutely over a four-month period and can occur during the first year of life. It is not necessarily associated with nonfunction of the involved kidney during excretory urography. Xanthogranulomatous pyelonephritis may be a segmental renal disease and may also occur bilaterally. Subtotal nephrectomy is the treatment of choice in these instances. Studies of calcium and phosphorus excretion in patients with this disease should be implemented in an e$ort to clarify the pathophysiologic aspects of the disease.
The urologist’s awareness of xanthogranulomatous pyelonephritis has increased over the past fifteen years because of a fourfold increase in the number of such cases reported from 1957 to the present.“’ At times xanthogranulomatous pyelonephritis may occur with unusual clinical manifestations that may preclude accurate preoperative diagnosis. This problem is compounded by the histologic similarity of this entity to renalcell carcinoma so that even the pathologic diagnosis can be confusing. 34 Our recent experience includes 5 cases with unique features which encompass a variety of diagnostic possibilities and perhaps offers new insight into the pathophysiologic aspects of this disease.
Case Reports Case 1 A twenty-four-year-old male was first seen in December, 1968, because of accelerated hypertension. At that time his blood pressure was 2SO/ 180 mm. Hg. Urographic and arteriographic evaluation revealed congenital absence of the right kidney. The left kidney appeared normal (Fig. 1A). Laboratory data included a blood urea nitro-
338
gen of 15 mg. per 100 ml. and a creatinine clearance of 108 cc. per minute. Four months later the patient was readmitted with the acute onset of headaches progressing to coma resulting from primary subarachnoid hemorrhage. There had been no interval history suggesting urinary infection. Over a two-week period resolution of neurologic deficits occurred, although a low-grade fever persisted. Following temperature elevation to 103” F., excretory urography demonstrated a space-occupying lesion in the upper pole of the left kidney which was shown to be avascular by selective arteriography (Fig. 1B). Urine cultures grew enterobacter. Following appropriate antibiotic therapy, left flank exploration revealed extensive local inflammation surrounding the upper pole of the kidney. Polar nephrectomy was performed. Gross inspection of the surgical specimen revealed sheets and cords of yellowish xanthomatous material which, on histologic examination, demonstrated large foamy macrophages interspersed with acute and chronic inflammatory cells typical of xanthogranulomatous pyelonephritis. Although his hypertension did not completely resolve, the patient did well postoperatively, and he was discharged with a blood urea nitrogen of 15 mg. per 100 ml.
UROLOGY
/ APRIL
1973
/ VOLUME
I, NUMBER4
FIGURE 1. Cuse 1. (A) Normal appearing left kidney visualized by selective angiography, Junuary, 1969. (B) Repeat left renal angiogram four months later demonstrutes stretching of upper pole arterial vessel.7 by avascular mass.
Case 2 A sixty-three-year-old female, previously in good health without prior history of urologic disease, was admitted with a five-week history of malaise, dull right upper-quadrant abdominal pain, and mild dysuria. Vomiting had occurred for several days prior to her admission. Physical examination revealed that she was in septic shock with a tender right upper-quadrant mass. Examination of the urine revealed microscopic pyuria and hematuria. Subsequent urine ~‘ultures were positive for Proteus mirabilis. Pertinent laboratory studies included a white blood cell count of 32,100 with a shift to the left, hemoglobin 8.9 Gm. per 100 ml., blood urea nitrogen 15 mg. per 100 ml., and creatinine 1.2 mg. per 100 ml. Excretory urography revealed poor function of the right kidney with multiple overlying caleifications. A ureteral catheter was passed to the renal pelvis, and gross pus was aspirated. Retrograde pyelography demonstrated extensive retroperitoneal abscess formation with fistulization to the small intestine (Fig. 2A). Nephrectomy was performed, the duodenal fistula was closed in
two layers, and the retroperitoneum was widely drained. The kidney and associated inflammatory tissues \veighed 543 Gm. Grossly, there was considerable loss of cortical tissue and dilatation of the collecting system. Thick nodules of yellowish tissue were visible adjacent to the renal pelvis. hlieroscopically, there was extensive acute and chronic inflammation with collections of large foamy macrol)hages tending to follow the outline of the calyceal system (Fig. 2B). Cultures of the kidney tissue revealed Proteus mirabilis. Postoperatively the patient’s course was marked by recurrent fistula followed by acute neerotizing colitis. Despite vigorous medical and surgical management, she died from overwhelming sepsis. Case 3 An eleven-month-old female was admitted for urologic evaluation because of a ten-month history of recurrent urinary tract infections unresponsive to repeated courses of antibiotic therapy. Excretory urography revealed deformity of the left kidney associated with a stenotic lesion
FIGURE 2. Case 2. (A) Retrogrude pyelography demonstrutes grossly distorted renal pelvis und pyeloduodenal jistula. (B) Representative microscopic section ofkidney reveals both chronic injiummatory cells und large macrophages with cleur cytoplasm, single nucleus, und no mitoses.
of the renal pelvis and tortuosity of the left ureter. Retrograde pyelography confirmed pelvic stenosis of a degree precluding calyceal filling. No vesicoureteral reflux was demonstrated. Physical examination was within normal limits. Urinalysis revealed microscopic pyuria. Subsequent cultures were positive for Klebsiella species. Evaluation for tuberculosis revealed negative findings. The blood urea nitrogen was 15 mg. per 100 ml.; the serum creatinine was 0.5 mg. per 100 ml. Although at surgery the kidney had a normal exploration of the renal external appearance, pelvis revealed almost complete stenosis which precluded operative repair. Nephrectomy was performed. On sectioning, areas of necrosis were observed within the lower pole of the kidney (Fig. 3). Histologically, there was acute and chronic inflammation interspersed with focal collections of foamy macrophages. Kidney cultures for tuberculosis were negative. Case
4
A forty-eight-year-old male entered the hospital with a four-month history of right flank pain and
340
progressive weight loss. Except for surgery at eleven years of age to remove bladder calculi, the patient denied any major illness or genitourinary problems. On physical examination a large, fixed, nontender mass was noted in the right upper quadrant, but no lymphadenopathy was noted. Laboratory examination revealed the following: hematocrit 26 per cent, white blood cell count 23,300, pyuria with Pseudomonas urinary tract infection, blood urea nitrogen 33 mg. per 100 ml., serum creatinine 2.3 mg. per 100 ml., and a creatinine clearance 22.5 ml. per minute. The serum calcium was 13.3 mg. per 100 ml., serum phosphorus 2.5 mg. per 100 ml., and serum alkaline phosphatase 15.8 Bodansky units. Urine calcium determination ranged from 16.6 to 18.4 of mg. per 100 ml., and tubular reabsorption phosphate of 50 per cent was markedly abnormal. Serum parathormone levels performed by radioimmune assay were in the low-normal range. Excretory urography demonstrated nonfunction of the right kidney with multiple radiopaque calculi. Selective arteriography outlined a small right renal artery with irregular intrarenal branches stretched around dilated calyces.
UROLOGY
/ APRIL 1973 / VOI,U~IE I, NUMBER 4
Tumor vessels were identified in the mid-kidney area (Fig. 4). Findings on inferior venacavography and metastatic skeletal survey, including hand films, were unremarkable. At the time of radical nephrectomy the mass was encased in yellow scar tissue adherent to the liver and colon. The specimen, covered by a thickened capsule, weighed 1,600 Gm., and no normal architecture was recognizable. Abscess areas with necrotic tissue were present throughout the mass. Microscopic sections revealed adenocarcinoma with perinephric extension and xanthogranulomatous pyelonephritis with renal lithiasis. Results of an assay of tumor tissue for parathormone were negative. Postoperatively, the serum calcium fell to 7.2 mg. per 100 ml. Case 5
This patient, a thirty-year-old female, was the recipient of a cadaver kidney transplant in September, 1969. She had had previoiis bilateral nephrectomy because of end-stage renal disease with hypertension. Following transplantation, a severe rejection reaction was treated vigorously with azathioprine (Imuran), prednisone, actinomycin D, and homograft irradiation. In addition she was given antilymphocytic globulin, and a thoracic duct fistula was created.
FIGURE 3. Case 3. Hemisected kidney shows silver probe in stenotic renul pelvis. Note ubscess present in lower pole. Cultures o.f kidney tissue .for tuberculosis were negative.
The transplant functioned until May, 1970, at which time she was admitted to the hospital with renal failure and urinary tract sepsis. Urine cultures grew Escherichia coli. Following emergency dialysis, the kidney was removed and weighed 230 Gm. Grossly, both the cortex and medulla were replaced with sheets of firm yellowwhite tissue. On microscopic examination these areas revealed acute inflammation coexistent with necrosis and collections of foamy macrophages typical of xanthogranulomatous pyelonephritis. Surprisingly little evidence of rejection was seen in the spared renal tissue. Comment The association of xanthogranulomatous pyelonephritis with chronic urinary tract infection The pathoa11d renal calculi is well established. logic features are also well defined and include (1) replacement of normal parenchyma by nodules of yellowish tissue; (2) widespread evidence of acute and chronic inflammation; (3) collections of large macrophages with foamy cytoplasm, a single nucleus, and no mitoses; and (4) frequent coexistence of abscess cavities.” Despite this clinical and pathologic information, the cause of this disease remains an enigma.’ Xanthogranulomatous pyelonephritis developed acutely and dramatically in an immunosuppressed patient (Case 5) resulting in homograft loss. The association of xanthogranulomatous pyelonephritis with hypernephroma (Case 4), as well as its development in an infant (Case 3), also suggest that the development of this disease may, at times, be re-
FIGURE 4. Case 4. Selective renal angiogram outlined typicul tumor vessels in midportion of right kidney. Intrarenul brunches over lower und middle poles ure stretched uround diluted culyceal system. Coexistent xanthogranulomatous pyelonephritis ond hypernephroma found in this kidney which also produced parathormone-like substunce.
341
lated to altered immune competence. We believe that this evidence should prompt careful investigation of serum immunoglobulins and cellular immune mechanisms in these patients. Most case reports emphasize a history of chronic urinary tract infection with xanthogranulomatous pyelonephritis occurring from the fifth to the seventh decades of life. The youngest case previously reported was in a seventeen-monthold child.” Case 3 demonstrates that occurrence within the first year of life is possible. The rapidity with which the pathologic changes of this disorder may develop is underscored by Case 1 in which the typical histologic pattern emerged within four months. These cases also emphasize the fact that xanthogranulomatous pyelonephritis is not necessarily associated with nonfunction of the involved kidney during excretory urography. Spontaneous pyeloduodenal fist& is rare. Although most reported cases have been associated with renal inflammation, the entity has not been previously described in association with xanthogranulomatous pyelonephritis.’ Fistulas, when previously reported in association with this disease, have been to the skin.‘sx In view of the widespread retroperitoneal inflammation in patients with xanthogranulomatous pyelonephritis, healing of a pyelointestinal fistula is impaired and represents a severe complication. Although there is a histologic resemblance between xanthogranulomatous pyelonephritis and renal-cell carcinoma, Case 4 is only the second reported case of the two diseases coexisting.” There are no previous reports of this entity associated with ectopic hormone production by a renal tumor. It might be postulated that the hypercalcemia observed in Case 4 resulted from the production of a parathormone-like substance by the hypernephroma and that calculous obstruction with development of xanthogranulomatous pyelonephritis occurred secondarily. The possibility of an underlying metabolic defect producing this disease has been previously raised.‘O Since 70 per cent of cases with this disorder have renal calculi, the careful study of calcium and phosphorous excretion by the kidneys in these patients appears justified. There is some precedent to this suggestion inasmuch as sarcoidosis, also a granulomatous disease, is associated with hypercalcemia and renal calculi.” Nephrectomy is the accepted treatment for xanthogranulomatous pyelonephritis. However,
342
this disease may occur in a solitary kidney (Case l), and bilateral renal involvement has been reported.” Our experience in Cases 1 and 3 confirms a previous report of segmental renal involvement, particularly in younger age groups.‘” If the affected kidney functions in part, and if there is damage to or absence of the contralateral kidney, partial nephrectomy is the favored treatment.“,x,l 1 Besides its diagnostic importance, selective renal angiography should be done prior to partial nephrectomy to provide a vascular map of the involved kidney.‘” This safe diagnostic modality greatly aids the surgeon and allows maximum conservation of renal parenchyma. University Hospital
of San Diego County 225 West Dickinson Street San Diego, California 92103 (DR. KAPLAN)
References 1. SELZE~, D. W., DAHLIN, D. C., and DEWEERU, J.H.: Tumefactive xanthogranulomatolls pyelonephritis, Surgery 42: 874 (1957). 2. ANHALT, RI.A., CAWOOD,C. D.,and SCOTT,R.: Acomprehensive review with report of 4 additional cases, J. Urol. 105: 10 (1971). 3. RIOS-DALENZ,J.L.,andPEACOCK,R. C.: Xanthogranulomatous pyelonephritis, Post-Grad. M. J. 45: 695 (1966). 4. MCKENZIE, K. R.: Xanthogranulomatons pyelonephritis: confusion with renal carcinoma, J. Ural. 92: 261 (1964). pyelonephritis, Irish 5. DINN, J. J.: Xanthogranulomatolls J. M. SC. 1: 431 (1968). and CHANDOR,S.B.: 6. CECCARELLI,F. E.,WUHSTER,J.C., Xanthogranulomatous pyelonephritis in an infant, J. Urol. 104: 755 (1970). 7. HOPKINS,W. F.,md PIENCE,J.hl.: Spontaneous pyeloduodenal fistula: a case report, ihid. 95: 489 (1966). Xanthogranulomatous pyelone8. DUTT, A. K., et al.: phritis, Post-Grad. hl. J. 45: 695 (1969). 9. ELLIOTT, C. B.,JOHNSON, H. W., and BALTohl,J.A.: Xanthogranulomatous pyelonephritis and perirenal xanthogrannlomata, Brit. J. Urol. 40: 548 (1968). 10. PARKER, J. M.: Xanthogranulomatous pyelonephritis, J. Urol. 96: 290 (1966). 11. MAYLOCK, R. L., BERTRAND, P.,MOHRISON, C. E.,and SCOTT, J. H.: Manifestations of sarcoidosis; analysis of 145 patients with a review of nine series selected from the literature, Am. J. Med. 35: 67 (1963). 12. ROSSI, P., MYERS, D. H., FUHEY, R., and BONFILSROBERTS, E. A.: Angiography in bilateral xanthogranulomatous pyelonephritis, Radiology 90: 320 (1968). 13. GRAI~IEH,L., and VARGAS, hl. A.: Xanthogranulomatous pyelonephritis in childhood, Am. J. Dis. Child. 123: 156 (1972). 14. PUIGVERT,A.,and GITTES,R. F.: Partial nephrectomy in the solitary kidney. I. Results in 10 cases of renal lithiasis, J. Urol. 100:238 (1968). 1Fj. I'INIK, Xl.,FREE]),T. A.,SMELLIE,W. A. B.,and WEID.su<,\V.: Xa~~tho~ra~lnlomatous pyelonephritis: angiographic considerations, Radiology 92: 537 (1968).
UROLOGY
/ APRIL 1973 / VOLUME I, NUMBER4
Clinical
J. H. MERING, G. W. KAPLAN,
PYELONEPHRITIS
Presentations
M.D. M.D.
A. P. MCLAUGHLIN,
III,
M.D.
From the Division of Urology, University Hospital of San Diego County, University of California, San Diego, California
ABSTRACTThe association of xanthogranulomatous pyelonephritis with renal-cell carcinoma and the loss of a kidney homograft because of this disorder suggest that in certain cases altered immunologic competence might be an etiologic factor in this disease. This entity may develop acutely over a four-month period and can occur during the first year of life. It is not necessarily associated with nonfunction of the involved kidney during excretory urography. Xanthogranulomatous pyelonephritis may be a segmental renal disease and may also occur bilaterally. Subtotal nephrectomy is the treatment of choice in these instances. Studies of calcium and phosphorus excretion in patients with this disease should be implemented in an e$ort to clarify the pathophysiologic aspects of the disease.
The urologist’s awareness of xanthogranulomatous pyelonephritis has increased over the past fifteen years because of a fourfold increase in the number of such cases reported from 1957 to the present.“’ At times xanthogranulomatous pyelonephritis may occur with unusual clinical manifestations that may preclude accurate preoperative diagnosis. This problem is compounded by the histologic similarity of this entity to renalcell carcinoma so that even the pathologic diagnosis can be confusing. 34 Our recent experience includes 5 cases with unique features which encompass a variety of diagnostic possibilities and perhaps offers new insight into the pathophysiologic aspects of this disease.
Case Reports Case 1 A twenty-four-year-old male was first seen in December, 1968, because of accelerated hypertension. At that time his blood pressure was 2SO/ 180 mm. Hg. Urographic and arteriographic evaluation revealed congenital absence of the right kidney. The left kidney appeared normal (Fig. 1A). Laboratory data included a blood urea nitro-
338
gen of 15 mg. per 100 ml. and a creatinine clearance of 108 cc. per minute. Four months later the patient was readmitted with the acute onset of headaches progressing to coma resulting from primary subarachnoid hemorrhage. There had been no interval history suggesting urinary infection. Over a two-week period resolution of neurologic deficits occurred, although a low-grade fever persisted. Following temperature elevation to 103” F., excretory urography demonstrated a space-occupying lesion in the upper pole of the left kidney which was shown to be avascular by selective arteriography (Fig. 1B). Urine cultures grew enterobacter. Following appropriate antibiotic therapy, left flank exploration revealed extensive local inflammation surrounding the upper pole of the kidney. Polar nephrectomy was performed. Gross inspection of the surgical specimen revealed sheets and cords of yellowish xanthomatous material which, on histologic examination, demonstrated large foamy macrophages interspersed with acute and chronic inflammatory cells typical of xanthogranulomatous pyelonephritis. Although his hypertension did not completely resolve, the patient did well postoperatively, and he was discharged with a blood urea nitrogen of 15 mg. per 100 ml.
UROLOGY
/ APRIL
1973
/ VOLUME
I, NUMBER4
FIGURE 1. Cuse 1. (A) Normal appearing left kidney visualized by selective angiography, Junuary, 1969. (B) Repeat left renal angiogram four months later demonstrutes stretching of upper pole arterial vessel.7 by avascular mass.
Case 2 A sixty-three-year-old female, previously in good health without prior history of urologic disease, was admitted with a five-week history of malaise, dull right upper-quadrant abdominal pain, and mild dysuria. Vomiting had occurred for several days prior to her admission. Physical examination revealed that she was in septic shock with a tender right upper-quadrant mass. Examination of the urine revealed microscopic pyuria and hematuria. Subsequent urine ~‘ultures were positive for Proteus mirabilis. Pertinent laboratory studies included a white blood cell count of 32,100 with a shift to the left, hemoglobin 8.9 Gm. per 100 ml., blood urea nitrogen 15 mg. per 100 ml., and creatinine 1.2 mg. per 100 ml. Excretory urography revealed poor function of the right kidney with multiple overlying caleifications. A ureteral catheter was passed to the renal pelvis, and gross pus was aspirated. Retrograde pyelography demonstrated extensive retroperitoneal abscess formation with fistulization to the small intestine (Fig. 2A). Nephrectomy was performed, the duodenal fistula was closed in
two layers, and the retroperitoneum was widely drained. The kidney and associated inflammatory tissues \veighed 543 Gm. Grossly, there was considerable loss of cortical tissue and dilatation of the collecting system. Thick nodules of yellowish tissue were visible adjacent to the renal pelvis. hlieroscopically, there was extensive acute and chronic inflammation with collections of large foamy macrol)hages tending to follow the outline of the calyceal system (Fig. 2B). Cultures of the kidney tissue revealed Proteus mirabilis. Postoperatively the patient’s course was marked by recurrent fistula followed by acute neerotizing colitis. Despite vigorous medical and surgical management, she died from overwhelming sepsis. Case 3 An eleven-month-old female was admitted for urologic evaluation because of a ten-month history of recurrent urinary tract infections unresponsive to repeated courses of antibiotic therapy. Excretory urography revealed deformity of the left kidney associated with a stenotic lesion
FIGURE 2. Case 2. (A) Retrogrude pyelography demonstrutes grossly distorted renal pelvis und pyeloduodenal jistula. (B) Representative microscopic section ofkidney reveals both chronic injiummatory cells und large macrophages with cleur cytoplasm, single nucleus, und no mitoses.
of the renal pelvis and tortuosity of the left ureter. Retrograde pyelography confirmed pelvic stenosis of a degree precluding calyceal filling. No vesicoureteral reflux was demonstrated. Physical examination was within normal limits. Urinalysis revealed microscopic pyuria. Subsequent cultures were positive for Klebsiella species. Evaluation for tuberculosis revealed negative findings. The blood urea nitrogen was 15 mg. per 100 ml.; the serum creatinine was 0.5 mg. per 100 ml. Although at surgery the kidney had a normal exploration of the renal external appearance, pelvis revealed almost complete stenosis which precluded operative repair. Nephrectomy was performed. On sectioning, areas of necrosis were observed within the lower pole of the kidney (Fig. 3). Histologically, there was acute and chronic inflammation interspersed with focal collections of foamy macrophages. Kidney cultures for tuberculosis were negative. Case
4
A forty-eight-year-old male entered the hospital with a four-month history of right flank pain and
340
progressive weight loss. Except for surgery at eleven years of age to remove bladder calculi, the patient denied any major illness or genitourinary problems. On physical examination a large, fixed, nontender mass was noted in the right upper quadrant, but no lymphadenopathy was noted. Laboratory examination revealed the following: hematocrit 26 per cent, white blood cell count 23,300, pyuria with Pseudomonas urinary tract infection, blood urea nitrogen 33 mg. per 100 ml., serum creatinine 2.3 mg. per 100 ml., and a creatinine clearance 22.5 ml. per minute. The serum calcium was 13.3 mg. per 100 ml., serum phosphorus 2.5 mg. per 100 ml., and serum alkaline phosphatase 15.8 Bodansky units. Urine calcium determination ranged from 16.6 to 18.4 of mg. per 100 ml., and tubular reabsorption phosphate of 50 per cent was markedly abnormal. Serum parathormone levels performed by radioimmune assay were in the low-normal range. Excretory urography demonstrated nonfunction of the right kidney with multiple radiopaque calculi. Selective arteriography outlined a small right renal artery with irregular intrarenal branches stretched around dilated calyces.
UROLOGY
/ APRIL 1973 / VOI,U~IE I, NUMBER 4
Tumor vessels were identified in the mid-kidney area (Fig. 4). Findings on inferior venacavography and metastatic skeletal survey, including hand films, were unremarkable. At the time of radical nephrectomy the mass was encased in yellow scar tissue adherent to the liver and colon. The specimen, covered by a thickened capsule, weighed 1,600 Gm., and no normal architecture was recognizable. Abscess areas with necrotic tissue were present throughout the mass. Microscopic sections revealed adenocarcinoma with perinephric extension and xanthogranulomatous pyelonephritis with renal lithiasis. Results of an assay of tumor tissue for parathormone were negative. Postoperatively, the serum calcium fell to 7.2 mg. per 100 ml. Case 5
This patient, a thirty-year-old female, was the recipient of a cadaver kidney transplant in September, 1969. She had had previoiis bilateral nephrectomy because of end-stage renal disease with hypertension. Following transplantation, a severe rejection reaction was treated vigorously with azathioprine (Imuran), prednisone, actinomycin D, and homograft irradiation. In addition she was given antilymphocytic globulin, and a thoracic duct fistula was created.
FIGURE 3. Case 3. Hemisected kidney shows silver probe in stenotic renul pelvis. Note ubscess present in lower pole. Cultures o.f kidney tissue .for tuberculosis were negative.
The transplant functioned until May, 1970, at which time she was admitted to the hospital with renal failure and urinary tract sepsis. Urine cultures grew Escherichia coli. Following emergency dialysis, the kidney was removed and weighed 230 Gm. Grossly, both the cortex and medulla were replaced with sheets of firm yellowwhite tissue. On microscopic examination these areas revealed acute inflammation coexistent with necrosis and collections of foamy macrophages typical of xanthogranulomatous pyelonephritis. Surprisingly little evidence of rejection was seen in the spared renal tissue. Comment The association of xanthogranulomatous pyelonephritis with chronic urinary tract infection The pathoa11d renal calculi is well established. logic features are also well defined and include (1) replacement of normal parenchyma by nodules of yellowish tissue; (2) widespread evidence of acute and chronic inflammation; (3) collections of large macrophages with foamy cytoplasm, a single nucleus, and no mitoses; and (4) frequent coexistence of abscess cavities.” Despite this clinical and pathologic information, the cause of this disease remains an enigma.’ Xanthogranulomatous pyelonephritis developed acutely and dramatically in an immunosuppressed patient (Case 5) resulting in homograft loss. The association of xanthogranulomatous pyelonephritis with hypernephroma (Case 4), as well as its development in an infant (Case 3), also suggest that the development of this disease may, at times, be re-
FIGURE 4. Case 4. Selective renal angiogram outlined typicul tumor vessels in midportion of right kidney. Intrarenul brunches over lower und middle poles ure stretched uround diluted culyceal system. Coexistent xanthogranulomatous pyelonephritis ond hypernephroma found in this kidney which also produced parathormone-like substunce.
341
lated to altered immune competence. We believe that this evidence should prompt careful investigation of serum immunoglobulins and cellular immune mechanisms in these patients. Most case reports emphasize a history of chronic urinary tract infection with xanthogranulomatous pyelonephritis occurring from the fifth to the seventh decades of life. The youngest case previously reported was in a seventeen-monthold child.” Case 3 demonstrates that occurrence within the first year of life is possible. The rapidity with which the pathologic changes of this disorder may develop is underscored by Case 1 in which the typical histologic pattern emerged within four months. These cases also emphasize the fact that xanthogranulomatous pyelonephritis is not necessarily associated with nonfunction of the involved kidney during excretory urography. Spontaneous pyeloduodenal fist& is rare. Although most reported cases have been associated with renal inflammation, the entity has not been previously described in association with xanthogranulomatous pyelonephritis.’ Fistulas, when previously reported in association with this disease, have been to the skin.‘sx In view of the widespread retroperitoneal inflammation in patients with xanthogranulomatous pyelonephritis, healing of a pyelointestinal fistula is impaired and represents a severe complication. Although there is a histologic resemblance between xanthogranulomatous pyelonephritis and renal-cell carcinoma, Case 4 is only the second reported case of the two diseases coexisting.” There are no previous reports of this entity associated with ectopic hormone production by a renal tumor. It might be postulated that the hypercalcemia observed in Case 4 resulted from the production of a parathormone-like substance by the hypernephroma and that calculous obstruction with development of xanthogranulomatous pyelonephritis occurred secondarily. The possibility of an underlying metabolic defect producing this disease has been previously raised.‘O Since 70 per cent of cases with this disorder have renal calculi, the careful study of calcium and phosphorous excretion by the kidneys in these patients appears justified. There is some precedent to this suggestion inasmuch as sarcoidosis, also a granulomatous disease, is associated with hypercalcemia and renal calculi.” Nephrectomy is the accepted treatment for xanthogranulomatous pyelonephritis. However,
342
this disease may occur in a solitary kidney (Case l), and bilateral renal involvement has been reported.” Our experience in Cases 1 and 3 confirms a previous report of segmental renal involvement, particularly in younger age groups.‘” If the affected kidney functions in part, and if there is damage to or absence of the contralateral kidney, partial nephrectomy is the favored treatment.“,x,l 1 Besides its diagnostic importance, selective renal angiography should be done prior to partial nephrectomy to provide a vascular map of the involved kidney.‘” This safe diagnostic modality greatly aids the surgeon and allows maximum conservation of renal parenchyma. University Hospital
of San Diego County 225 West Dickinson Street San Diego, California 92103 (DR. KAPLAN)
References 1. SELZE~, D. W., DAHLIN, D. C., and DEWEERU, J.H.: Tumefactive xanthogranulomatolls pyelonephritis, Surgery 42: 874 (1957). 2. ANHALT, RI.A., CAWOOD,C. D.,and SCOTT,R.: Acomprehensive review with report of 4 additional cases, J. Urol. 105: 10 (1971). 3. RIOS-DALENZ,J.L.,andPEACOCK,R. C.: Xanthogranulomatous pyelonephritis, Post-Grad. M. J. 45: 695 (1966). 4. MCKENZIE, K. R.: Xanthogranulomatons pyelonephritis: confusion with renal carcinoma, J. Ural. 92: 261 (1964). pyelonephritis, Irish 5. DINN, J. J.: Xanthogranulomatolls J. M. SC. 1: 431 (1968). and CHANDOR,S.B.: 6. CECCARELLI,F. E.,WUHSTER,J.C., Xanthogranulomatous pyelonephritis in an infant, J. Urol. 104: 755 (1970). 7. HOPKINS,W. F.,md PIENCE,J.hl.: Spontaneous pyeloduodenal fistula: a case report, ihid. 95: 489 (1966). Xanthogranulomatous pyelone8. DUTT, A. K., et al.: phritis, Post-Grad. hl. J. 45: 695 (1969). 9. ELLIOTT, C. B.,JOHNSON, H. W., and BALTohl,J.A.: Xanthogranulomatous pyelonephritis and perirenal xanthogrannlomata, Brit. J. Urol. 40: 548 (1968). 10. PARKER, J. M.: Xanthogranulomatous pyelonephritis, J. Urol. 96: 290 (1966). 11. MAYLOCK, R. L., BERTRAND, P.,MOHRISON, C. E.,and SCOTT, J. H.: Manifestations of sarcoidosis; analysis of 145 patients with a review of nine series selected from the literature, Am. J. Med. 35: 67 (1963). 12. ROSSI, P., MYERS, D. H., FUHEY, R., and BONFILSROBERTS, E. A.: Angiography in bilateral xanthogranulomatous pyelonephritis, Radiology 90: 320 (1968). 13. GRAI~IEH,L., and VARGAS, hl. A.: Xanthogranulomatous pyelonephritis in childhood, Am. J. Dis. Child. 123: 156 (1972). 14. PUIGVERT,A.,and GITTES,R. F.: Partial nephrectomy in the solitary kidney. I. Results in 10 cases of renal lithiasis, J. Urol. 100:238 (1968). 1Fj. I'INIK, Xl.,FREE]),T. A.,SMELLIE,W. A. B.,and WEID.su<,\V.: Xa~~tho~ra~lnlomatous pyelonephritis: angiographic considerations, Radiology 92: 537 (1968).
UROLOGY
/ APRIL 1973 / VOLUME I, NUMBER4