Br. J. Surg. Vol. 62 (1975) 563-564
Xanthogranulomatous pyelonephritis: a report of 2 cases 0. S . M O R G A N , G . D . N I C H O L S O N , P . S J O H N S O N A N D D. W . A T K I N S O N * SUMMARY
Two cases o f xanthogranulomatous pyelonephritis are reported in Nezroes. The clinical findings are similar to those in previously reported cases. In 1 case nephvolithotomy was jollowed by the development of’ a perinephric abscess 8 months later. Late diagnosis has limited experience with more conservative management, which should be reserved .for demonsfrahly ,focal disease.
XANTHOGRANULOMATOUS pyelonephritis is an infrequently occurring variant of pyelonephritis rarely reported in Negroes. In a review of 38 cases in whom ethnic origin was noted only 10 per cent occurred in Negroes (Anhalt et al., 1971). We report 2 cases of this condition in patients of African descent, in one of whom management by removal of the associated renal calculus was unsuccessful.
Fig. 1. Cose 1. Photograph of the bisected kidney showing the grossly dilated caliceal system. The renal parenchyma is almost indistinguishable, being replaced by yellow nodules. The arrow points to the c a l c ~ l u sin a lower pole calix.
Case reports Case 1 : A 67-year-old housewife was admitted to the University Hospital, Kingston, after an 8-week period of increasing ill-health. During the previous 4 months she had experienced intermittent episodes of pain in the left flank and iliac fossa, accompanied by increased frequency of micturition. In the 4 weeks antedating admission she had complained of lassitude, chills, rigors and night sweats. She had lost over 13 kg in weight since the onset of her illness. On examination a large non-tender irregular mass was palpablein the right hypochdndrium.Theurinecontained many pus cells and a few red blood cells, but was sterile on culture. The haemoglobin was 8.6 g/100 ml, the white cell count 8100/mm? with a normal differential, and the ESR 100mm in 1 hour. Blood cultures were sterile. The blood urea was 28 mg/lOO ml and the plasma creatinine 1.0 mg/100 ml. An abdominal film revealed a large soft tissue mass in the left upper quadrant with a dense opacity within its borders. Intravenous pyelography showed only faint excretion of the dye on the left side. The left renal outline was enlarged and calicectasis was observed in the upper pole. No caliceal pattern was distinguishable in the lower pole which contained a large calculus. The right kidney functioned normally. At cystoscopy there was milky urine coming from the left ureter, whose orifice was grossly oedematous. A left retrograde pyelogram confirmed the presence of calicectasis. Pus obtained at this examination was sterile. At nephrectomy a large hydronephrotic kidney was found. The perinephric tissues were grossly oedematous and the kidney was adherent to the spleen and descending colon. The kidney was 17 cm long and weighed 300 g. The calices were enormously distended by thin pus and there was fibrotic thickening of their walls. There was severe loss of renal parenchymal tissue. A large calculus 3 cm in diameter lay within a lower pole calix. Multiple yellow nodules measuring up to 2 cm in diameter were scattered throughout the solid areas of tissue (Fig. 1). The histological appearances were of acute on chronic pyelonephritis with widespread ranthogranulomatous change. The renal parenchyma was almost entirely destroyed apart from a few remnant tubules containing hyaline casts. There was extensive fibrosis and scattered abscess formation. Thc dense
Fig. 2. Cow 1. Photomicrograph showing lipid-laden histiocytcs. ( Y 210.)
infiltration of inflammatory cells included neutrophils, plasma cells and lymphocytes with follicle formation. I n addition. abundant foamy histiocytes were present, often forming large aggregates. Oil-Red 0 stains confirmed these cells to be rich in cytoplasmic lipid. Very few giant cells were found (F;g. 2). Case 2 : A 51-year-old housewife was transferred to the University Hospital. Kingston, for investigation of a nonfunctioning left kidney. A large c a l c ~ l uhad ~ been removed from the left renal pelvis 8 months previously. Two months prior to admission she had been treated for a urinary tract infection and 2 weeks later had developed a left perinephric abscess which, although widely incised and drained, had continued to discharge. On examination the patient seemed surprisingly well. She was afrebile. Large quantities of foul-smelling pus could bc expressed from the left loin wound and yielded a mixed growth
* Departments of Medicine, Surgery and Pathology. University of the West Indies. Mona. Kingston 7 , Jamaica, West Indies.
0. S. Morgan et al. of Staphylococcus pyogenes, Proteus sp. and Pseudonioiicis pyocyanea. Intravenous pyelography showed a non-functioning left kidney; the right kidney was normal. Retrograde pyelography was unsuccessful. At operation a large pyonephrosis was found. The kidney was removed with difficulty from the surrounding tissues to which it was extremely adherent. The kidney was 12 cm long and weighed 150 g. The calices, which were dilated, had thickened walls and haemorrhagic mucosa. The renal parenchyma was greatly reduced, pale in colour and contained multiple yellow nodules of variable size, averaging 1 cm in diameter, and frequently bordering the calices. No calculi were found. There was severe acute on chronic pyelonephritis with extensive destruction of renal parenchyma and abscess formation. The inflammatory infiltrate contained numerous neutrophils, plasma cells and lymphocytes with follicle formation. Many foamy histiocytes forming large aggregates were present. These cells were rich in cytoplasmic lipid. An occasional multinucleate giant cell was also present.
Discussion These cases are similar to those reported previously, in which the prominent features include an enlarged non-functioning kidney associated with renal calculi, obstruction and chronic infection. The interest in this condition lies in the importance of its differentiation from carcinoma and tuberculosis of the kidney on clinical, radiological and pathological grounds, permitting a planned approach to therapy. Malek et al. (1972) have suggested the classification of xanthogranulomatous pyelonephritis into three stages. Our cases fall into stage 111 of this classification, with nephric, perinephric and paranephric involvement in which removal of the diseased kidney is the treatment of choice. The disease does not tend to recur after nephrectomy even following incomplete excision (Elliott et al., 1968; Malek et al., 1972), and this strongly suggests the pre-eminence of local rather than metabolic factors in its causation.
564
Anhalt et al. (1971) treated one of their patients with rare bilateral disease by nephrolithotomy with a favourable outcome. Our second case was treated by nephrolithotomy at a time when the diagnosis was unsuspected. The subsequent development of a perinephric abscess is indicative of persistent infection in that kidney. Diffuse involvement of the affected kidney has commonly preceded diagnosis and this has limited attempts at conservative management. Nephrolithotomy, alone or combined with partial excision, should be confined to the focal form of the disease in which absence of function is confined to the abnormal portion of the kidney (Mitchell et al., 1959). Non-function of the whole kidney, particularly where the associated calculus lies in a polar calix, should alert one to the possibility of diffuse disease. Until earlier diagnosis becomes possible, attempts at local excision will remain exceptional and total nephrectomy the established procedure for diffuse disease.
References
c. D. and SCOTT R . (1971) Xanthogranulomatous pyelonephritis : a comprehensive review with report of 4 additional cases. J . Urol. 105, 10-17. ELLIOTT C. B., JOHNSON H. w. and BALFOUR J. A. (1968) Xanthogranulomatous pyelonephritis and perirenal xanthogranuloma. Br. J . Urol. 40, 548555. MALEK R . s., GREENE L. F., DEWEERD J. H . and FARROW G . M . ( 1972) Xan thogranulomatous pyelonephri tis. Br. J . Urol. 44, 296-308. MITCHELL R . E., DOBSON A . I. and KAY s. (1959) Xanthogranulomatous pyelonephritis. Am. Pruct. Dig. Treat. 10, 2150-2155.
ANHALT M. A., CAWOOD
Xanthogranulomatous pyelonephritis: a report of 2 cases 0. S . M O R G A N , G . D . N I C H O L S O N , P . S J O H N S O N A N D D. W . A T K I N S O N * SUMMARY
Two cases o f xanthogranulomatous pyelonephritis are reported in Nezroes. The clinical findings are similar to those in previously reported cases. In 1 case nephvolithotomy was jollowed by the development of’ a perinephric abscess 8 months later. Late diagnosis has limited experience with more conservative management, which should be reserved .for demonsfrahly ,focal disease.
XANTHOGRANULOMATOUS pyelonephritis is an infrequently occurring variant of pyelonephritis rarely reported in Negroes. In a review of 38 cases in whom ethnic origin was noted only 10 per cent occurred in Negroes (Anhalt et al., 1971). We report 2 cases of this condition in patients of African descent, in one of whom management by removal of the associated renal calculus was unsuccessful.
Fig. 1. Cose 1. Photograph of the bisected kidney showing the grossly dilated caliceal system. The renal parenchyma is almost indistinguishable, being replaced by yellow nodules. The arrow points to the c a l c ~ l u sin a lower pole calix.
Case reports Case 1 : A 67-year-old housewife was admitted to the University Hospital, Kingston, after an 8-week period of increasing ill-health. During the previous 4 months she had experienced intermittent episodes of pain in the left flank and iliac fossa, accompanied by increased frequency of micturition. In the 4 weeks antedating admission she had complained of lassitude, chills, rigors and night sweats. She had lost over 13 kg in weight since the onset of her illness. On examination a large non-tender irregular mass was palpablein the right hypochdndrium.Theurinecontained many pus cells and a few red blood cells, but was sterile on culture. The haemoglobin was 8.6 g/100 ml, the white cell count 8100/mm? with a normal differential, and the ESR 100mm in 1 hour. Blood cultures were sterile. The blood urea was 28 mg/lOO ml and the plasma creatinine 1.0 mg/100 ml. An abdominal film revealed a large soft tissue mass in the left upper quadrant with a dense opacity within its borders. Intravenous pyelography showed only faint excretion of the dye on the left side. The left renal outline was enlarged and calicectasis was observed in the upper pole. No caliceal pattern was distinguishable in the lower pole which contained a large calculus. The right kidney functioned normally. At cystoscopy there was milky urine coming from the left ureter, whose orifice was grossly oedematous. A left retrograde pyelogram confirmed the presence of calicectasis. Pus obtained at this examination was sterile. At nephrectomy a large hydronephrotic kidney was found. The perinephric tissues were grossly oedematous and the kidney was adherent to the spleen and descending colon. The kidney was 17 cm long and weighed 300 g. The calices were enormously distended by thin pus and there was fibrotic thickening of their walls. There was severe loss of renal parenchymal tissue. A large calculus 3 cm in diameter lay within a lower pole calix. Multiple yellow nodules measuring up to 2 cm in diameter were scattered throughout the solid areas of tissue (Fig. 1). The histological appearances were of acute on chronic pyelonephritis with widespread ranthogranulomatous change. The renal parenchyma was almost entirely destroyed apart from a few remnant tubules containing hyaline casts. There was extensive fibrosis and scattered abscess formation. Thc dense
Fig. 2. Cow 1. Photomicrograph showing lipid-laden histiocytcs. ( Y 210.)
infiltration of inflammatory cells included neutrophils, plasma cells and lymphocytes with follicle formation. I n addition. abundant foamy histiocytes were present, often forming large aggregates. Oil-Red 0 stains confirmed these cells to be rich in cytoplasmic lipid. Very few giant cells were found (F;g. 2). Case 2 : A 51-year-old housewife was transferred to the University Hospital. Kingston, for investigation of a nonfunctioning left kidney. A large c a l c ~ l uhad ~ been removed from the left renal pelvis 8 months previously. Two months prior to admission she had been treated for a urinary tract infection and 2 weeks later had developed a left perinephric abscess which, although widely incised and drained, had continued to discharge. On examination the patient seemed surprisingly well. She was afrebile. Large quantities of foul-smelling pus could bc expressed from the left loin wound and yielded a mixed growth
* Departments of Medicine, Surgery and Pathology. University of the West Indies. Mona. Kingston 7 , Jamaica, West Indies.
0. S. Morgan et al. of Staphylococcus pyogenes, Proteus sp. and Pseudonioiicis pyocyanea. Intravenous pyelography showed a non-functioning left kidney; the right kidney was normal. Retrograde pyelography was unsuccessful. At operation a large pyonephrosis was found. The kidney was removed with difficulty from the surrounding tissues to which it was extremely adherent. The kidney was 12 cm long and weighed 150 g. The calices, which were dilated, had thickened walls and haemorrhagic mucosa. The renal parenchyma was greatly reduced, pale in colour and contained multiple yellow nodules of variable size, averaging 1 cm in diameter, and frequently bordering the calices. No calculi were found. There was severe acute on chronic pyelonephritis with extensive destruction of renal parenchyma and abscess formation. The inflammatory infiltrate contained numerous neutrophils, plasma cells and lymphocytes with follicle formation. Many foamy histiocytes forming large aggregates were present. These cells were rich in cytoplasmic lipid. An occasional multinucleate giant cell was also present.
Discussion These cases are similar to those reported previously, in which the prominent features include an enlarged non-functioning kidney associated with renal calculi, obstruction and chronic infection. The interest in this condition lies in the importance of its differentiation from carcinoma and tuberculosis of the kidney on clinical, radiological and pathological grounds, permitting a planned approach to therapy. Malek et al. (1972) have suggested the classification of xanthogranulomatous pyelonephritis into three stages. Our cases fall into stage 111 of this classification, with nephric, perinephric and paranephric involvement in which removal of the diseased kidney is the treatment of choice. The disease does not tend to recur after nephrectomy even following incomplete excision (Elliott et al., 1968; Malek et al., 1972), and this strongly suggests the pre-eminence of local rather than metabolic factors in its causation.
564
Anhalt et al. (1971) treated one of their patients with rare bilateral disease by nephrolithotomy with a favourable outcome. Our second case was treated by nephrolithotomy at a time when the diagnosis was unsuspected. The subsequent development of a perinephric abscess is indicative of persistent infection in that kidney. Diffuse involvement of the affected kidney has commonly preceded diagnosis and this has limited attempts at conservative management. Nephrolithotomy, alone or combined with partial excision, should be confined to the focal form of the disease in which absence of function is confined to the abnormal portion of the kidney (Mitchell et al., 1959). Non-function of the whole kidney, particularly where the associated calculus lies in a polar calix, should alert one to the possibility of diffuse disease. Until earlier diagnosis becomes possible, attempts at local excision will remain exceptional and total nephrectomy the established procedure for diffuse disease.
References
c. D. and SCOTT R . (1971) Xanthogranulomatous pyelonephritis : a comprehensive review with report of 4 additional cases. J . Urol. 105, 10-17. ELLIOTT C. B., JOHNSON H. w. and BALFOUR J. A. (1968) Xanthogranulomatous pyelonephritis and perirenal xanthogranuloma. Br. J . Urol. 40, 548555. MALEK R . s., GREENE L. F., DEWEERD J. H . and FARROW G . M . ( 1972) Xan thogranulomatous pyelonephri tis. Br. J . Urol. 44, 296-308. MITCHELL R . E., DOBSON A . I. and KAY s. (1959) Xanthogranulomatous pyelonephritis. Am. Pruct. Dig. Treat. 10, 2150-2155.
ANHALT M. A., CAWOOD
