Misleading leads: focal xanthogranulomatous pyelonephritis in childhood.
Xanthogranulomatous pyelonephritis is a morphologic variant of pyelonephritis. Focal disease is very rare and can be misdiagnosed.
SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Xanthogranulomatous pyelonephritis is a morphologic variant of pyelonephritis. Focal disease is very rare and can be misdiagnosed.
Xanthogranulomatous pyelonephritis (XGP) is an unusual chronic inflammatory condition which most often affects women in their 5th to 7th decades and is rare in infants. Predisposing factors include infection, calculi and obstructive uropathy. We have reviewed the surgical files of 4 cases seen over a 28 yr period from 1964-91. All of the 3 partial and one total nephrectomy specimens demonstrated typical features of XGP with renal parenchyma effaced by a mixed acute and chronic inflammatory infiltrate which included prominent aggregates of foamy histiocytes containing eosinophilic inclusions. No Michaelis-Gutmann bodies were seen. This study shows the association of XGP with chronic infection, anatomical malformation and reflux in infancy, and raises the possibility of a temporary altered immune response in its pathogenesis.
Xanthogranulomatous pyelonephritis is a rare disease and rare cause of suppurative proliferative pseudotumor in children. An accurate diagnosis is difficult because of its clinical, radiological, sonographical and pathologic-anatomical similarities to renal tuberculosis. After removing the diseased kidney the prognosis is excellent. A 6-year old boy with xanthogranulomatous pyelonephritis is reported.
BACKGROUND: Xanthogranulomatous pyelonephritis is a severe, atypical form of chronic renal parenchymal infection accounting for 6/1000 surgically proved cases of chronic pyelonephritis. Its manifestations mimic those of neoplastic and other inflammatory renal parenchymal diseases and, consequently, it is often misdiagnosed preoperatively. AIM: To examine the relation between clinical history and the results of renal investigations performed in children with xanthogranulomatous pyelonephritis. METHOD: A retrospective review of 31 cases presenting with the histopathological diagnosis of xanthogranulomatous pyelonephritis between 1963 and 1999. RESULTS: The mean follow up was 8.2 years. The male:female ratio was 1:1.1. The left kidney was affected in 26 of the 31 patients. The positive findings on examination and investigation at presentation were: fever, 16 children; pyuria, 26 children; positive urine culture, 16 children. A haemoglobin of < 100 g/l was measured in 27 of 31 patients and 15 of 18 patients tested had a raised erythrocyte sedimentation rate of > 20 mm in the first hour. Twenty six children had renal calculi, with a large reduction in the function of the affected kidney on isotope scintigraphy in 27 of the 29 patients tested. Hypertrophy of the contralateral kidney, shown on imaging, was present in 17 of 31 patients. CONCLUSIONS: Increasing awareness of this condition should lead to the diagnosis being suspected preoperatively.
Xanthogranulomatous pyelonephritis is an uncommon form of chronic aggressive infection of the kidney and surrounding tissues usually affecting middle aged women. It is rarely seen in childhood. We report the case of a female child with a history of fatigue, progressive anorexia, and fever in whom a diagnosis of pyelonephritis on a predisposing factor of lithiasis was suggested. After initial antibiotic treatment, nephrectomy was needed for an acute general deterioration. Pathology demonstrated xanthogranulomatous pyelonephritis.
Xanthogranulomatous pyelonephritis in native and allografted kidneys is a rare variant of severe chronic infection of the renal parenchyma. In a native kidney the diagnosis may sometimes be established by ultrasonography and computed tomography. In the renal allograft, the diagnosis could only be established by histologic evaluation of the transplant biopsy or nephrectomy. The reported case presents a febrile patient with a failing renal graft, in whom xanthogranulomatous pyelonephritis was established by histologic evaluation of transplantectomy specimens. Xanthogranulomatous pyelonephritis should therefore be included in the list of possible etiologies of the fever in patient with nonfunctioning transplanted kidney.
Four cases of xanthogranulomatous pyelonephritis, treated at the Department of Urology of the Military Medical Academy, are described. Focal lesions of the kidney were found in all of them. A precise pre- and intraoperative diagnosis is impossible or doubtful because the clinical symptoms and macroscopic appearance of the involved kidney suggest the presence of a tumour. Treatment consists mainly in nephrectomy.
BACKGROUND AND PURPOSE: Xanthogranulomatous pyelonephritis (XGP) is a severe, chronic renal-parenchymal infection. Nephrectomy is the treatment of choice. Because of the renal and perirenal inflammatory changes that commonly accompany XGP, the laparoscopic approach is difficult. We compared our experience with laparoscopic and open surgical nephrectomy for XGP. PATIENTS AND METHODS: A retrospective chart review of all adult nephrectomy specimens with the pathologic diagnosis of XGP between January 1997 and May 2003 was performed. Preoperative presentation, operative details, and postoperative recovery and complications were included in the data collection. RESULTS: Three patients approached laparoscopically and eight patients approached with open surgery were found to have XGP on pathologic analysis. The disease was suspected preoperatively in all patients. Among the laparoscopically treated patients, there was 1 (33%) who suffered major complications; this was the only patient who required conversion to open surgery. Among the open-surgical group, there were 2 (22%) major and 3 (33%) minor complications. Postoperative hospitalization was longer in the open-surgical group (mean 13.7 v 4.7 days), and when the case of open conversion was excluded, narcotic use was less in the laparoscopy group. CONCLUSIONS: The treatment of some XGP cases with laparoscopic nephrectomy is a possible, albeit challenging, option. The incidences of intraoperative and postoperative complications were roughly equivalent in the laparoscopic and open-surgery patients in our study. If completed, laparoscopy appears to be associated with decreased postoperative morbidity. However, this may represent selection bias, and larger, prospective studies may better define the suspected benefit.
Four patients with xanthogranulomatous pyelonephritis were found to have renocolic fistulae. Coincidentally, the left kidney was involved in all four cases. All patients presented with renal mass. Two cases have had coexistent renal stones, one of them presented with massive upper gastrointestinal bleeding as a result of portal hypertension. Another patient had a history of Schistosomiasis. In none of the patients was the renal condition confidently diagnosed preoperatively, nor was the colonic fistula suspected. In all four patients, nephrectomy was performed together with resection of the involved colon followed by a satisfactory recovery. The possibility of a colonic fistula should be kept in mind as a complication to this rare renal condition in spite of the absence of colonic symptoms and normal finding in barium enema studies.
The association of xanthogranulomatous pyelonephritis (XPN) and systemic amyloidosis is extremely rare. This association has been described in only six cases. We present a 4-year-old male admitted with a history of anuria and abdominal pain. Investigations revealed multiple calculi in both kidneys. A right pyelolithotomy and left nephrectomy were performed. Histological examination demonstrated XPN and amyloidosis. At discharge serum creatinine had dropped to 1.1 mg/dl but after being lost to follow up for 9 years, the child was readmitted because of edema. Laboratory examination revealed a nephrotic syndrome and serum creatinine of 2.3 mg/dl. Rectal biopsy showed the presence of amyloid. A treatment by colchicine was unsuccessful. Fifteen months later, at the age of 15 years, the patient developed terminal renal failure (serum creatinine 14 mg/dl).
We report the case of a 68 year-old woman who underwent surgical treatment for xanthogranulomatous pyelonephritis. Xanthogranulomatous pyelonephritis is a rare, chronic, inflammatory lesion of the kidney. It is most frequently seen in middle-aged women. Because of its clinical and radiological similarities to other renal lesions it can be difficult to establish a preoperative diagnosis. Nephrectomy is needed in most patients.
Xanthogranulomatous pyelonephritis in children, contrary to adult onset, rarely is associated with non-function or calcification. The lesion is predominantly on the left side in children. There appears to be a normal humoral but temporarily impaired cellular immune response in addition to sustained depression of polymorphonuclear chemotaxis. The etiology of this is uncertain but may be attributed partially to hyperosmolarity of serum and urine, and to leukocyte specific antinuclear antibodies. The presence of leukocyte specific antinuclear antibody or cold agglutining may interfere with normal phagocyte chemotaxis requiring tissue macrophages to produce a xanthogranulomatous reaction to bacterial invasion.
A 47-year-old female was admitted with colicky pain in the left flank. Ultrasonography and abdominal CT revealed left perirenal hematoma. Abdominal CT revealed a multilocular cystic lesion in the upper pole of the left kidney. Renal angiography revealed left renal cell carcinoma. Left nephrectomy was performed under a tentative diagnosis of left renal cell carcinoma. However, pathological diagnosis was xanthogranulomatous pyelonephritis. Xanthogranulomatous pyelonephritis is an atypical form of chronic renal parenchymal infection that can be classified as diffuse type or focal type. Preoperative diagnosis of focal xanthogranulomatous pyelonephritis is difficult because of clinical and radiological similarities to renal cell carcinoma. Our case is presented with a brief review of the literature.
Two unusual cases of xanthogranulomatous pyelonephritis studied by both magnetic resonance imaging and computed tomography, are presented. Extension of the disease depicted by both computed tomography and magnetic resonance imaging was compatible with the findings at surgery. Computed tomography seems to be sufficient for xanthogranulomatous pyelonephritis imaging evaluation, while magnetic resonance imaging is not recommended on a routine basis, since no additional valuable information is yielded.
INTRODUCTION: Xanthogranulomatous pyelonephritis is a morphologically and clinically unique manifestation of chronic pyelonephritis with the formation of pus or granulomas. The most frequent predisposing factors for the development of xanthogranulomatous pyelonephritis are urinary obstruction (e. g., stones, tumors, congenital anomalies and functional impairment) and infection of the collecting system. CASE REPORT: We describe a 2-year-old female patient with unclear abdominal complaints, diarrhea, malaise, loss of appetite, weight loss, pale skin color, and recurrent and undulating fever in the presence of known left nephrolithiasis. Based on the clinical examination and imaging, above all, CT, the presumptive diagnosis of xanthogranulomatous pyelonephritis of the left kidney was made. A left lumbar nephrectomy was performed and histology confirmed the diagnosis. CONCLUSION: Xanthogranulomatous pyelonephritis is a relatively rare entity that is associated with obstruction (e. g., stones) and infection of the urinary tract. Its rarity and resultant unfamiliarity often delay diagnosis and therapy, which in turn affect the prognosis. Furthermore, this entity can be mistaken for renal tumors (renal cell carcinoma and Wilms tumor), but nowadays this should be mostly eliminated with the advances in the imaging methods.
Xanthogranulomatous pyelonephritis is an uncommon disease in children. We report of a 2-month-old girl with urinary tract infection and with a renal mass detected by ultrasound scan. The preoperative differential diagnoses were Wilms tumor, multicystic dysplastic kidney, renal abscess, and mesoplastic nephroma. The subsequent histopathological findings allowed the diagnosis of xanthogranulomatous pyelonephritis (XGP). Although XGP is rare at this age, it must be considered in the differential diagnosis of a child presenting with renal mass, either with or without associated urolithiasis, anemia, and elevated inflammatory markers. The long-standing mainstay of therapy for diffuse XGP has been nephrectomy. However, we report the successful preservation of renal mass with partial nephrectomy for diffuse XGP. The affected kidney grew normally and preserved residual function after the operation. Hence we strongly suggest parenchymal saving in pediatric cases of XGP to preserve renal function.
We observed xanthogranulomatous pyelonephritis in 5 of 27 nephrectomies (18.5%) performed for end stage pyelonephritis. Two patients were 5 months old or younger, of normal birth weight and the products of uncomplicated full-term pregnancies. In all cases clinical histories and findings were consistent with xanthogranulomatous pyelonephritis yet this diagnosis was suspected prospectively in only 1 case. Our observations, supported by recent literature, suggest that xanthogranulomatous pyelonephritis may occur more commonly than previously reported. In addition, the clinical and pathological expression of this process in the pediatric population appears to be no different from that in adults. The true incidence and presumed chronicity of this process need to be reevaluated in light of recent data.
Xanthogranulomatous pyelonephritis is an uncommon chronic inflammatory renal disorder. Most cases have been described in middle aged women and it is extremely rare in children. We report a case of a 4 year old girl who suffered from recurrent urinary tract infection and antibiotic therapy resistance. She had low growth-rate and palpable left renal mass on examination and was confirmed by radiological findings. We performed left kidney partial resection and then histological examination showed focal xanthogranulomatous pyelonephritis. The focal form of the disease may respond to antibiotic treatment although usually an enucleation or partial resection must be performed. In conclusion xanthogranulomatous pyelonephritis should be considered in the differential diagnosis of a renal mass and recurrent urinary tract infection in childhood.