Case Reports © 1990 S. Karger AG, Basel 0302-2838/90/0181-0074Î2.7 5/0

Eur Urol 1990;18:74-76

Xanthogranulomatous Pyelonephritis Associated with Renal Cell Carcinoma

1608155

Report on Two Cases and Review of the Literature

I. Papadopoulos, B. Wirth, H. Wand Department of Urology, University of Kiel, FRG

Key Words. Xanthogranulomatous pyelonephritis • Renal cell carcinoma Abstract. Xanthogranulomatous pyelonephritis is a rare and particularly aggressive variant of chronic destructive pyelonephritis. Even when all modem diagnostic possibilities are exhausted, it is often not possible to distinguish xanthogranulomatous pyelonephritis from a renal cell carcinoma preoperatively. In clinical practice false diagnoses are therefore frequent. The coexistence of xanthogranulomatous pyelonephritis and renal cell carcinoma is extremely rare. We report 2 such cases. In 1 case surgery was performed on the kidney affected by xanthogranulomatous pyelonephritis using a renal sparing technique.

Introduction Xanthogranulomatous pyelonephritis (XGP), first described by Schlagenhaufer [1] in 1916, is a special form of severe chronic infec­ tion of the renal parenchyma. It is possible that it develops as the result of an unexplained pathological immune reaction to bacterial toxins [2]. XGP resembles renal cell carcinoma, so that due to the preoperative difficulties nephrectomy is performed in the majority of cases. Histologically XGP is characterized by the typical lipid-laden macrophages (xanthoma cells). The coexistence with renal cell car­ cinoma has rarely been reported. To date only 9 cases have been published in the world literature [3-10]. We report 2 patients with XGP and simultaneous renal cell car­ cinoma, 1 in the same kidney and the other in the contralateral kidney.

Slight leukocyturia and erythrocyturia were demonstrable in the uri­ nary sediment; the urine culture was sterile. On ultrasound a solid tumor was seen at the upper pole of the right kidney (fig. 1). An intravenous urogram showed a round solidified mass at the lateral border of the right kidney with calcifications. CT showed a partially calcified, cystic structure at the upper pole of the right kidney (fig. 2). Selective renal angiography revealed a suspicious, weakly vascularized area lateral to the upper pole of the kidney with faintly recognizable atypical vessels and prolonged retention of contrast medium (fig. 3). A renal cell carcinoma was suspected, and the right kidney was exposed. Intraoperatively a cystic tumor, about 5 cm in diameter, with a thick wall was found. It was unclear whether its calcified, amorphous content was malignant or not. Nephrectomy was per­ formed. Pathohistologically the excised kidney showed the picture of a highly differentiated renal cell cancer and XGP. The postoper­ ative course was uneventful.

Case Reports Case 2 M.H., a 60-year-old woman, underwent urological treatment for urinary retention. Previously she had had no complaints at all. Sig­ nificant prior illnesses included diabetes mellitus, hypertension and a goiter. The patient was obese, and on admission she was in a reduced general condition. Both kidneys were painless. There was no fever. Laboratory tests showed an elevated sedimentation rate of 72/121 mm, leukocytosis (22,000/pl) and elevated y-glutamyltransferase (41 U/l). Urethrocystoscopically a large area with pseudopolyps was found at the roof and back wall of the bladder. The histological evaluation of the biopsy specimen showed ‘eosinophilic urocystitis’ with no suspicion of malignancy. Downloaded by: University of Exeter 144.173.6.94 - 5/5/2020 2:14:04 PM

Case 1 A.K., a 69-year-old man. 3 months prior to hospital admission, a tumor was diagnosed on the optic nerve of the left eye, which was a metastasis from an unknown tumor. While searching for the pri­ mary tumor, a mass on the right kidney was observed. The patient had no specific complaints or symptoms. Among the other condi­ tions affecting the patient, diabetes mellitus should be mentioned. On admission the patient was in a good general condition. There was no tenderness on palpation of either kidney, and no mass was palpable in the flank. Body temperature and blood pressure were normal. Laboratory tests showed all blood counts to be within normal limits, aside from an elevated sedimentation rate of 20/50 mm.

Xanthogranulomatous Pyelonephritis and Renal Cell Carcinoma

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Fig. 1. Sonogram of kidney. Solid tumor at the upper pole of the right kidney. Fig. 2. CT scan. Calcified cystic structure at the upper pole of the right kidney. Fig. 3. Selective renal angiography. Suspicious, only weakly vas­ cularized mass lateral to the upper pole.

Discussion XGP is a variant of chronic interstitial pyelonephritis. Histologi­ cally it is characterized by typical xanthoma cells. The frequent occur­ rence of XGP in the female sex is related to the greater frequency of chronic pyelonephritis in women. Children can also be affected, although confusion with a Wilms’ tumor is possible [11-14]. The etiology of XGP is still unclear. A number of factors, such as urinary tract obstruction in connection with inadequately treated chronic urinary tract infections [2], lymphatic or venous blockage, abnormal lipid metabolism [ 15] and an impaired immune response [13] seem to play an important role. Nephrolithiasis of many years duration, primary hyperparathyroidism and alcohol abuse also ap­ pear to be predisposing factors in the development of the disease. Hypertension is found in 12-45% of the patients [2, 16, 17], dia­ betes mellitus in 7% [18]. Of our 2 cases both had diabetes mellitus and the 2nd patient additionally had hypertension. Association with renal cell carcinoma [3-10] and transitional cell carcinoma [19-21] has only rarely been described. The clinical symptoms of XGP are uncharacteristic: kidney pains, episodes of fever, loss of appetite, weight loss and severe malDownloaded by: University of Exeter 144.173.6.94 - 5/5/2020 2:14:04 PM

Renal ultrasound revealed a solid tumor at the left kidney and a partially solid, partially cystic tumor at the upper edge of the right kidney. In the intravenous urogram, a mass was seen in the left kidney and a second questionable mass at the upper pole of the right kidney. A CT scan showed a large tumor in the left kidney with involve­ ment of the renal veins and two flat masses at the upper and lower poles of the right kidney. Renal angiography showed a large tumor with pathological vas­ cularity on the left side. On the right side, the tumors could not be seen on the angiogram. Bilateral kidney tumors were suspected, and both kidneys were exposed transperitoneally. Intraoperatively a large tumor was found in the left kidney. Nephrectomy was performed, including resection of the enlarged parahilar lymph nodes. In the right kidney two para­ nephritic abscesses were found at the upper and lower poles, and they were extirpated. The histological examination revealed a renal cell carcinoma in the left kidney infiltrating the renal capsule and growing into the renal vein but without lymph node metastasis (T3b) and in the right kidney XGP. With antibiotic treatment, the postop­ erative course was uneventful.

Papadopoulos/Wirth/Wand

aise. The physical examination often reveals a palpable tumor, flank pain and in rare cases a cutaneous fistula. The typical pathological laboratory findings include an elevated sedimentation rate and a moderate degree of leukocytosis. Elevated hepatic enzyme levels have been described in a quarter of the patients. They are generally reversible postoperatively. In bacterio­ logical investigations Escherichia coli, Proteus mirabilis, Pseudo­ monas aeruginosa and Klebsiella predominate. The sonographic, CT and X-ray findings (intraveneous urog­ raphy, angiography) are not specific. They often reveal tumors that are definitely compatible with a renal cell carcinoma. The ultra­ sound pattern of the XGP foci corresponds approximately to that of a solid mass and often has an inhomogeneous echo. In an intravenous urogram the kidneys often appear enlarged. Calculi, tumor-like structures, deformations of the renal calyces and calcified intraparenchymal structures can often be recognized. CT shows tumorous lesions, which generally cannot be distin­ guished from renal cell carcinoma, calcifications and often exten­ sion of the inflammatory process into perirenal spaces. With mag­ netic resonance imaging, infiltration of the inflammatory tumor into adjacent tissue structures (colon, spleen, psoas, abdominal wall) can be demonstrated very well [22]. The angiographic findings in XGP show vessels stretched around a hypovascular or avascular tumor, sometimes pathological vessels, but rarely with AV shunts [2], In most cases these masses cannot be distinguished with complete certainty from renal cell car­ cinoma [11,22]. Although all diagnostic possibilities have been exhausted, in most cases it is not possible to make a correct preoperative diagno­ sis. Theoretically it would be possible to perform percutaneous fineneedle aspiration under ultrasound guidance to exclude the possibil­ ity of renal cell carcinoma and to make sure of the preoperative diagnosis. In practice, however, this would not be particularly help­ ful, because usable material is rarely aspirated [2, 12, 17]. Therefore the final diagnosis is made intraoperatively or postoperatively on the basis of histology. In the treatment of XGP conservative management is indicated only in cases that are inoperable or when an operation is refused. Such cases are, however, likely to be protracted, and severe compli­ cations may occur [17]. In the diffuse form of XGP, the most fre­ quent form, nephrectomy is the treatment of choice. In the rare cases of segmental involvement an attempt should be made to use renal sparing techniques. Surgery can be a very demanding matter, from the technical point of view, because of the extensive inflamma­ tory infiltration. If XGP is suspected, preoperative antibiotic treat­ ment should be given. The prognosis is good after the affected kidney has been removed, provided the contralateral kidney functions normally. There have, however, been reports of postoperative complications, in the form of intestinal fistulae or recurrences of urosepsis [2].

References 1 Schlagenhaufer F: Über eigentümliche Staphylomykosen der Nieren und des pararenalen Bindegewebes. Z Pathol (Frankfurt) 1916;19:139-148. 2 Malek RS, Edler JS: Xanthogranulomatous pyelonephritis: A critical analysis of 26 cases and of the literature. J Urol 1978; 119:589-593.

3 Schoborg TW, Saffos RO, Urdaneta L, Lewis CW: Xanthogran­ ulomatous pyelonephritis associated with renal cell carcinoma. J Urol 1980;124:125-127. 4 Radin DR, Chandrasoma P: Coexistent xanthogranulomatous pyelonephritis and renal cell carcinoma. J Comput Tomogr 1987;11:294-196. 5 Prati GF, Muoio A, Recchid G, et al: Association of xanthogran­ ulomatous pyelonephritis and renal cancer. Arch Esp Urol 1987; 40:608-609. 6 Elliott CB, Johnson HW, Balfour JA: Xanthogranulomatous pyelonephritis and perirenal xanthogranuloma. Br J Urol 1968; 40:548-555. 7 Mering JH, Kaplan GW, McLaughlin AP: Xanthogranuloma­ tous pyelonephritis: Unusual clinical presentations. Urology 1973;1:338-342. 8 Lorentzen M, Overgaard Nielsen H: Xanthogranulomatous py­ elonephritis. Scand J Urol Nephrol 1980;14:193-200. 9 Goulding FJ: Letter to the editor. J Urol 1981 ; 125:268. 10 Piscioli F, Luciani L: Association of xanthogranulomatous py­ elonephritis with small renal cell carcinoma. Eur Urol 1984; 10: 62-66. 11 Gammill S, Rabinowitz JG, Peace R: New thoughts concerning xanthogranulomatous pyelonephritis (XGP). Am J Roentgenol 1975;125:154-163. 12 Swoboda PM, Schrott KM: Die xanthogranulomatöse Pyelo­ nephritis (XGP) bei Kindern und Erwachsenen. Urologe A 1985;24:20-24. 13 Tolia BM, Iloreta A, Freed SZ, et al: Xanthogranulomatous pyelonephritis: Detailed analysis of 29 cases and a brief discus­ sion of atypical presentations. J Urol 1981;126:437-442. 14 Watson AR, Marsden HB, Lendon M, Morris-Jones PH: Renal pseudotumors caused by xanthogranulomatous pyelonephritis. Arch Dis Child 1982;57:635-637. 15 Hooper RG, Kempson RL, Schlegel JU: Xanthogranulomatous pyelonephritis. J Urol 1962:88:585-593. 16 Goldman SM, Hartman DS, Fishman EK, et al: CT of xantho­ granulomatous pyelonephritis: Radiologic-pathologic correla­ tion. Am J Roentgenol 1984;142:963-969. 17 Flynn JT, Molland EA, Paris AMI, Blandy JP: The underesti­ mated hazards of xanthogranulomatous pyelonephritis. Br J Urol 1979;51:443-444. 18 Vahlensieck W Jr, Wimmer B, Westenfelder M: Diagnostik und Therapie der xanthogranulomatösen Pyelonephritis. Urologe A 1988;27:184-189. 19 Jeney D, Gemünd R, Körte H: Xanthogranulomatöse Pyelo­ nephritis mit Nierenbeckenkarzinom: Eine seltene Koinzidenz. Urologe A 1986;25:220-223. 20 McDonald GSA: Xanthogranulomatous pyelonephritis. J Pathol 1981;133:203-213. 21 Godec CJ, Murrah VA: Simultaneous occurrence of transitional cell carcinoma and urothelial adenocarcinoma associated with xanthogranulomatous pyelonephritis. Urology 1985;26:412-415. 22 Feldberg MAM, Driessen LP, Witkamp TD, et al: Xanthogran­ ulomatous pyelonephritis: Comparison of extent using com­ puted tomography and magnetic resonance imaging in one case. Urol Radiol 1988;10:92-94. Dr. I. Papadopoulos Department of Urology, University of Kiel Amold-Heller-Strasse 7, D-2300 Kiel (FRG) Downloaded by: University of Exeter 144.173.6.94 - 5/5/2020 2:14:04 PM

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Xanthogranulomatous pyelonephritis associated with renal cell carcinoma. Report on two cases and review of the literature.

Xanthogranulomatous pyelonephritis is a rare and particularly aggressive variant of chronic destructive pyelonephritis. Even when all modern diagnosti...
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