Review literature: (Bingol-Kologlu et al, 2002) (Zorzos et al, 2003).
Definition of Xanthogranulomatous Pyelonephritis
Xanthogranulomatous pyelonephritis (XP) is a rare form of chronic pyelonephritis with foam cells (large macrophages with foamy lipid-containing cytoplasm), which lead to kidney destruction.
XP is rare (incidence 1.4/100000), women are more likely affected than men.
XP is usually a unilateral renal disease, which develops in nonfunctioning kidneys with nephrolithiasis, urinary tract infection and upper urinary tract obstruction. The most commonly isolated bacterial organisms are E. coli, Proteus, Klebsiella and Staphylococcus.
Pathology of of Xanthogranulomatous Pyelonephritis
Enlarged kidney, enlarged renal pelvis filled with pus (pyonephrosis), orange-yellow inflammatory lesions in addition to tissue necrosis and small abscesses. The renal involvement is limited initially to segments of the kidney, later the inflammatory process involves most of the kidney. Hereby, the renal parenchyma is replaced by a granulomatous inflammatory tissue and loses its function. The disease may spread into adjacent structures of the retroperitoneum.
The inflammatory tissue contains neutrophils, lymphocytes, plasma cells, necrosis, and large macrophages with foamy lipid-containing cytoplasm. Sometimes epitheloid granulomas are found.
Signs and Symptoms of Xanthogranulomatous Pyelonephritis
- Flank pain (70%)
- Fever and chills (70%)
- Flank tumor
- Persistent bacteriuria
- General malaise and weight loss.
Urine sediment shows leukocyturia, bacteriuria, and possibly the detection of foam cells (lipid-laden macrophages).
Urine culture has a low specifity, but often positive.
Renal ultrasound shows an enlarged kidney with a mixed echogenic mass, it can not be reliably distinguished from a malignant tumor. Nephrolithiasis may be seen.
KUB x-ray may show an increased renal shadow and nephrolithiasis. Urogram reveals in 30-80% a missing renal function with no opacification of the affected side.
CT is the diagnostic tool of choice. It shows a lesion of the kidney with inhomogeneous contrast enhancement, reduced kidney function, hydronephrosis and detection of nephrolithiasis. Often, a renal cell carcinoma cannot be ruled out.
MRI is an alternative to CT, especially in renal failure.
To clarify the remaining kidney function, when organ preservation seems possible.
Differential diagnosis of Xanthogranulomatous Pyelonephritis
Renal cell carcinoma: the diagnosis of xanthogranulomatous pyelonephritis if often made via the specimen after surgery for suspected RCC.
Treatment of Xanthogranulomatous Pyelonephritis
Standard therapy for xanthogranulomatous pyelonephritis is nephrectomy or partial nephrectomy. Surgery is difficult because of adhesions, a laparoscopic approach is not recommended. Perioperative antibiotic therapy is important, which may be modified after the results of intraoperative swab tests. In individual cases, it was possible to cure segmental XP with antibiotic therapy and internal splinting.
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Bingol-Kologlu u.a. 2002 BINGOL-KOLOGLU, M. ;
CIFTCI, A. O. ; SENOCAK, M. E. ; TANYEL, F. C. ;
KARNAK, I. ; BUYUKPAMUKCU, N.:
- Xanthogranulomatous pyelonephritis in children: diagnostic and
In: Eur J Pediatr Surg
12 (2002), Nr. 1, S. 42–8
Zorzos u.a. 2003 ZORZOS, I. ; MOUTZOURIS, V. ;
KORAKIANITIS, G. ; KATSOU, G.:
- Analysis of 39 cases of xanthogranulomatous pyelonephritis with
emphasis on CT findings.
In: Scand J Urol Nephrol
37 (2003), Nr. 4, S. 342–7