Case Report Urol Int 1992;48:453-456

Fulvio Di Torinoa Giuseppe Capizzia Lucio Laurinia Giuseppe Costaa Riccardo Zennarib Eugenio Dall’Orsoc Walter Artibanid Dino Lavellia

Focal Xanthogranulomatous Pyelonephritis: Diagnostic and Therapeutic Aspects

Departments of Urology, Radiology and Pathology; Institute of Urology, University of Padua. ULSS 20 Regione Veneto, Camposampiero (Padova), Italy

K e y w o rd s

A b stra ct

Xanthogranulomatous pyelonephritis Computed tomography Preoperative diagnosis Histologic extemporary examination Conservative surgery

The authors report 2 cases of localized xanthogranulomatous pyelonephritis (XGP) in which computed tomography (CT) permitted to raise a motivated clinical suspicion of inflammatory disease: the surgical exploration docu­ mented absence of neoplasm and allowed a conservative therapy implying only the removal of the mass. A literature review confirms that some CT signs in XGP permit differentiation from carcinoma. If they are present, in unifocal cases of disease, the authors suggest a limited surgical approach and a thera­ peutic strategy as conservative as possible.

Xanthogranulomatous pyelonephritis (XGP) is an atypical and infrequent [1.2] form of renal bacterial infec­ tion. It is possible to recognize it in a localized form (iso­ lated and single renal mass) and a diffuse one (with multi­ ple lesions all over the kidney) [3, 4], The disease is usually unilateral and more common in women between 50 and 70, but it may often concern both male sex [4-6] and pediatric age [7], Its name comes from the typical microscopical features characterized by macrophages, ‘foamy’ in their aspect because of the phagocytosis of the lipids and cholesterol liberated from tissue necrosis [8]. Even if quite rare in its occurrence, XGP has a particular interest for the urologist in consequence of its hard preop­ erative differentiation from renal neoplasms [9]. ‘Tradi­

Received: August 27, 1990 Accepted alter revision: May 27. 1991

tional' methods (urography, arteriography), as well as so­ nography [10, 11 ]. do not seem to be resolutive. A qualitative progress, about the XGP instrumental diagnosis, has been introduced adopting computed-tomography (CT) as a common way to study renal masses showed by X-rays or sonography [12-15], but even mag­ netic resonance (MR), which is becoming more wide­ spread, seems as reliable as CT [16. 17]. Nevertheless, total nephrectomy is often the only available therapy, independently of the size, because of the difficulty to obtain a definite preoperative diagnosis. This research starts with the analysis of 2 cases in which the inflammatory nature of renal lesions ‘solid’ at the CT has been preoperatively suspected. Such a pre­ sumptive diagnosis has been confirmed during the surgi­ cal exploration by extemporary histology and it permitted

Dotl. Fulvio Di Tonno Divisione Urológica. Ospcdale P. C'osma Via P. Cosma I 1-35012 Camposampiero (Padua) (Italy)

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in trod uction

in both cases the removal of the only mass, saving the renal unit concerned. The definitive pathologic examina­ tion showed XGP. The objective of this study is the attempt to codify, even if in an indicative way and concerning selected cases, some standard of CT diagnosis and of XGP surgical ther­ apy to avoid, whenever possible, an undue nephrectomy.

Case Reports Case I A 49-year-old woman had developed l month before acute left flank pain, hyperpyrexia and macrohematuria. On admission to our Hospital she presented normal blood tests, urine analysis docu­ mented hematopyuria and urine culture revealed Escherichia coli. Sonography showed an inhomogeneous left kidney mass, 4-5 cm in diameter, with a central colliquation area. CT scan (fig. 1) showed a reduced enlargement of the left kidney including, in the lower pole, a round area with a density showing intermediate values between ‘sol­ id’ and ‘liquid’; it was inhomogeneous at the bottom due to the pres­ ence of septa, with a thickened Gcrota’s fascia, some impressed peripheral calyx, and slight deformation of renal contour. Suspecting a possible inflammatory nature of the mass, it was decided to con­ tinue surgically by a lumbar approach, having the purpose of a con­ servative treatment if extemporary histology and the extension of the mass made it possible. Intraoperatively, the lesion, not visible on the external surface of the kidney, looked yellowish, with a maximum diameter of 5 cm and had well-defined contours with a precise cleav­ age plane. A'cuneiform' biopsy was made and it was sent to be sub­ jected to extemporary histological examination. The result was nega­ tive as regards to neoplasm and the mass was removed, not without difficulty, until reaching the healthy parenchyma with margins cer­ tainly free from disease. The final pathological examination report attested XGP presence. The postoperative course resulted in being regular. One year later the patient enjoys excellent health and the radiological and clinical check-up was negative.

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Fig. 2. Case 2: appearance on CT scan (see the text).

Case 2 A 50-year-old women on admission to our Center complained about hyperpyrexia and left flank pain; she presented anemia and urine analysis showed hematopyuria. After an appropriate antibiotic therapy, urography demonstrated a mass deforming the left lower renal pole with calices dislocation and parcnchymographic aspect compatible with ‘solid’ lesion. The sonography visualized an inho­ mogeneous left renal mass, with thick walls and coarse septa inside. CT scan (fig. 2) showed a left kidney quite enlarged in toto and the presence in the lower pole of an intraparenchymatous mass deform­ ing the renal contour, with a low density (nearer to ‘liquid’ values), inhomogeneous, approximately 5 cm in diameter, with a thick peripherical ring deeply imbued with contrast medium and not welldefined contours, crossing into a fat capsule which did not show defi­ nite contours and looked inhomogeneous and thicker than usual. In the lower part of the mass, underlined coarse septa were evident, Gerota’s fascia was thickened, and the excretory system moderately impressed. Suspecting a benign inflammatory lesion, it was decided to continue with lumbotomy: the mass revealed itself immediately under the external surface of the kidney and looked yellowish and with a maximum diameter of 5 cm. It was difficult to isolate it from the surrounding parenchyma and the extemporary ‘cuneiform’ biopsy resulted in being negative as regards to neoplasm. The mass was removed and the incision was run in order to leave margins with laudable parenchyma, well distinguishable from the removed lesion. The definitive histological examination documented XGP diagnosis. The patient, after a regular postoperative course, enjoys good health and no clinical signs of relapse.

Com m ent

At present, CT is the most effective instrument for the diagnosis of renal masses. In 1984. Hertle et al. [12], on the basis of the retrospective analysis of 2 patients sub­ jected to nephrectomy with preoperative diagnosis of renal carcinoma in 1 case and of inflammatory lesion in

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Xanthogranulomatous Pyelonephritis

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Fig. 1. Case l : appearance on CT scan (see the text).

the other, codified the existence of six peculiar signs of XGP, namely: (1) calculi; (2) global renal enlargement; (3) spread of infiltration into fat capsule; (4) thickening of Gerota’s fascia; (5) multifocal areas of varying density, and (6) characteristic difference of density between cortex and medulla as a result of a reduced contrast medium concentration in the pyramids (only after angiography). Even in both our cases, CT documented the presence of some peculiar signs, so that we think it is necessary tak­ ing into account the experience of Hertle et al. [12], to analyze them one by one. The evenly distributed renal enlargement (sign 2 in Hertle’s chart) seems to be a quite typical sign of paren­ chymal phlogistic disease because tumors generally alter roughly the renal contour with a clear delimination from the renal laudable parenchyma. The spreading of the infil­ tration into the capsule fat (sign 3 in Hertle's chart) and a thickening of Gerota’s fascia (sign 4 in Hertle’s chart) are themselves meaningful criteria of differential diagnosis. The presence of a casual hypodense and slightly inhomo­ geneous area is suggestive of a diganosis of an inflamma­ tory lesion, especially if it concerns an enlarged kidney (we can compare this specification with sign 5 in Hertle’s chart). The presence within hypodense areas of roughly, regular septa, considerably thicker than the ones of cysts with septa or multilocular cysts, without calcifications and even in thickness and density different from those present in the malignant neoplastic forms represent, ac­ cording to us, further diagnostic information. This is really important for a diagnosis of phlogistic lesion which presents abscesses deliminiated by connective septa. Confronted with these radiological signs and with a clinical history of septic disease, we thought it right to per­ form a surgical lumbar exploration in the motivated clini­ cal suspicion of a single inflammatory benign lesion, although we could not exclude the presence of a neoplas­ tic lesion. The positive biopsy made possible a conserva­ tive therapy, saving a healthy renal unit.

D iscussion

new and difficult therapeutic problems. There is now a spectrum of renal masses, solid, liquid or intermediate, i.e., oncocytoma [10], angiomyolipoma [19], hematoma [20], abscesses and anthrax [21,22], with different biolog­ ical behavior conditions and therapeutic strategies rang­ ing from radical demolition to ‘nephron-sparing’ surgery. The focal XGP. subject of our study, gives diagnostic problems that are difficult to resolve. On one hand the clinical features are unimportant, on the other the inhomogeneous or solid aspect on CT analysis is often indistinguishable from the one of a renal carcinoma. It is then necessary to emphasize the importance of a careful anamnesis and, even more, of a minute analysis of the CT documentation to get to know a foreseeble diagnosis, hav­ ing a subsequent rational planning of the therapeutic strategy. Clinical ‘flair" and some ‘subtle’ CT signs can raise a preoperative diagnostic suspicion and avoid the planning of undue radical nephrectomies. The hypothesis that CT may give important specification in XGP diagno­ sis seems to be confirmed by the retrospective analysis of the 2 cases we have described. In both of them we observed the presence of some aspects which are de­ scribed by Hertle (signs 2, 3, 4 and, approximately, 5). Moreover, we pointed out as meaningful the presence of regular septa within inhomogeneous hypodense areas in­ cluded in renal parenchyma. These reports have to be considered characteristic but not pathognomonic. The whole of them do not authorize predetermined certitudes at all, but it may lead to a reasonable and calculable XGP diagnosis. Then, we think that in the presence of a medium to small focal lesion (not bigger than half a kid­ ney) a surgical ‘limited’ approach and an attitude as con­ servative as possible, including the removal of the only mass, represent the most suitable choice. In our experi­ ence, lumbotomy revealed itself conform to purpose. However, this attitude is made possible by close team­ work with a pathologist, by a macroscopic intraoperative examination and by an extemporary microscopic one as accurate as possible; the qualifying objective of this diag­ nostic-therapeutic strategy is the saving of a functioning renal unit.

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The finding of a renal mass, incidental or suspected, makes a urologist confronted with exact, difficult choices. The correct interpretation of the nature of the lesion and a proper therapeutic approach have a vital importance for the patient’s future. The recent introduction of new methods has given an important aid to differential diagnostic of renal masses, but the improvement of diagnostic possibilities involved

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R eferences

Focal xanthogranulomatous pyelonephritis: diagnostic and therapeutic aspects.

The authors report 2 cases of localized xanthogranulomatous pyelonephritis (XGP) in which computed tomography (CT) permitted to raise a motivated clin...
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