Introduction: Xanthogranulomatous pyelonephritis (XGPN) is an atypical form of chronic pyelonephritis referred to as the ‘‘great imitator’’ because the clinical and radiological findings closely resemble other pathological entities, especially for the focal forms. Distinguishing focal XGPN from renal cancer is preoperatively difficult. Methods: We report a total number of 12 pseudotumoural XGPN cases diagnosed and treated in our department. The aim of this study is to try to better understand the clinicopathological profile of XGPN and improve its management. Results: The mean age of patients was 51.52 years. Gender ratio was 0.71. An obstructive renal calculus was noticed in nine patients (75%). Only one patient (8.3 %) presented with loin pain associated with fever, weight loss, asthenia, and increased biological inflammation markers. A bifocal mass was noticed in one case (8.3 %). The average size of the tumour was 6.58 cm. The mass was cystic in three cases (25 %). Perinephral fat strand, thickening of Gerota’s fascia, hydronephrosis, and presence of renal calculi was noticed in all solid tumour cases. XGPN was suspected in only one case (8.3%), a percutaneous biopsy showed XGPN lesions treated by antibiotics and a double J drainage. Radical nephrectomy was performed in eight patients (66.7 %) and three patients underwent partial nephrectomy (25 %). No recurrence of XGPN has been noted. Conclusion: Pseudotumoural XGPN is a rare benign disease of the kidney. Its treatment should be conservative. Lack of knowledge of this disease may explain the high rate of abusive nephrectomies. These data should be considered in the future.

/pdf/xanthogranulomatous-pyelonephritis-presenting-as-a-2s69rw9kjt.pdf

Xanthogranulomatous pyelonephritis presenting as a pseudotumour.

Xanthogranulomatous pyelonephritis (XPN) is an unusual and severe form of chronic inflammatory lesion of the kidney, characterised by the destruction of the renal parenchyma and the presence of multinucleated giant cells and lipid-laden macrophages, inflammatory infiltration and intensive renal fibrosis. There are a few cases in the literature which describe the disease in children. The pathomechanism of XPN is poorly understood. Renal obstruction with concomitant urinary tract infection is the most commonly associated pathological finding. The process is typically unilateral and may be focal or diffuse. In both cases, the perirenal infiltration is possible and can be mistaken for common renal neoplasm or inflammatory process. The symptoms are non-specific. Diagnostic imaging techniques with clinical suspicion have enabled XPN to be diagnosed and differentiated from malignancy with a high degree of confidence. Computed tomography (CT) is the mainstay of diagnostic imaging. The definitive diagnosis of XPN is based on pathological assessment after nephrectomy. We review and illustrate the clinical, radiological, surgical and pathological characteristics of XPN in children. All cases shown are surgically and histopathologically proven. • XPN can present different clinical manifestations.
 • CT is the mainstay of diagnostic imaging in XPN.
 • Focal type of XPN should be included in the differential diagnosis of children with a renal mass.
 • There are no clear guidelines on the management of XPN.
 • Conservative and surgical treatments should be considered for each individual case.
 • Histopathological examination confirms the diagnosis and excludes other benign and malign diseases.

/pdf/xantogranulomatous-pyeloneprhritis-in-children-2cg356v27d.pdf

Xantogranulomatous pyeloneprhritis in children

Xanthogranulomatous pyelonephritis (XGP) is a rare renal tumor that arises as a complication of chronic obstructive pyelonephritis of uncertain etiology. It is primarily an adult tumor seen occasionally in children associated with urinary tract obstruction due to congenital urological anomalies, nephrolithiasis, and recurrent urinary tract infections. Radiologically, it may show neoplastic features such as those seen in common pediatric renal malignancies like wilms’ tumor and renal cell carcinoma. This overlap in radiological manifestation frequently leads to misdiagnosis and delay in appropriate intervention. We report a case of a 3 years old boy who presented with history of recurrent urinary tract infections and a left renal mass initially thought to be Wilms’ tumor. We present a case of a 3 years old boy admitted to the Pediatric oncology unit at Muhimbli National Hospital in Dar es Salaam, Tanzania with one year history of recurrent fever and urinary tract infection signs and symptoms refractory to antibiotic therapy. He was eventually found to have a left kidney mass detected at the District hospital by abdominal ultrasound performed to evaluate a flank mass that was felt by his mother. He was then referred to our unit for a suspicion of Wilms’ tumor which finally turned out  to be a left kidney Xanthogranulomatous pyelonephritis. He underwent a successful left nephrectomy and was discharged from hospital in a stable clinical condition and remains asymptomatic at the time of submission of this case report. This case report underscores the need for clinicians attending a febrile child with a renal mass that can be confused with common pediatric renal malignancies such as Wilms’ tumor to broaden their differential diagnosis. The case also underlines the significance of individualized patient evaluation because this patient would have otherwise received preoperative chemotherapy under the International Society of Pediatric Oncology (SIOP) guidelines if the diagnosis of Wilms tumor was not ruled out.

/pdf/case-report-xanthogranulomutous-pyelonephritis-presenting-as-30lk9myblk.pdf

Case report: Xanthogranulomutous pyelonephritis presenting as "Wilms' tumor".

Xanthogranulomatous pyelonephritis-mimicking renal mass in 5-month-old child.

Xanthogranulomatous pyelonephritis (XGPN) is a very rare, unusual variant of pyelonephritis characterized by destruction of renal parenchyma. It usually occurs in adults with a history of recurrent urinary tract infections. The condition is rare in children and the disease can imitate renal tumors. Here, we describe a 12-year-old boy who presented with abdominal pain. He did not have any history of urinary tract infection. Computed tomography and magnetic resonance imaging showed a cystic lesion in the left upper kidney. The patient underwent radical nephrectomy with a provisional diagnosis of Wilms tumor however histopathological examination of specimen revealed XGPN. Xanthogranulomatous pyelonephritis should be kept in mind in the differential diagnosis of renal lesions in childhood, during surgery if any suspicion from the diagnosis, a frozen biopsy should have been taken.

A case of xanthogranulomatous pyelonephritis mimicking Wilms tumor.

Shah K et al. Bilateral Focal Xanthogranulomatous Pyelonephritis ... Eur J Pediatr Surg 2011; 21: 207 – 208 Introduction ▼ Xanthogranulomatous pyelonephritis (XGPN) is a distinct, severe, atypical and chronic renal parenchymal infection. It is encountered predominantly in middleaged women and is relatively rare in childhood [1] . XGPN occurs predominantly unilaterally, and bilateral disease is extremely rare [2 – 6] . It may present either as diff use or, less commonly, as a focal infection. This uncommon condition is characterized by invasion to surrounding structures. Its resemblance to tumoral features presents a diagnostic and therapeutic challenge. Nephrectomy is usually indicated for unilateral XGPN, and the prognosis is excellent if treated early [1] . However, the management of XGPN is diffi cult due to the risk of permanent renal dysfunction. We report a rare case of bilateral focal XGPN in a child presenting with a complex renal mass managed non-surgically.

Bilateral focal xanthogranulomatous pyelonephritis in a child presenting as complex cystic renal mass: a report on non-surgical treatment.

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiographic entity of heterogenous etiologies, which are grouped together because of similar findings on neuroimaging studies, associated with similar symptom complex of headache, vision loss, altered mentation, and seizures. In this report, we describe a case of PRES in setting of postobstructive diuresis in a 5-year-old male child, whose solitary functioning kidney was obstructed by a 1.6-cm radio-opaque stone, who after percutaneous nephrostomy (PCN) diversion developed persistent hypocalcemia which persisted despite maximum replacement by iv calcium gluconate drip, and the child developed repeated generalized tonic clonic convulsions and became unconscious for 4 days. Computerized tomography (CT) scan of the brain showed typical hypodensities in bilateral occipitoparietal regions suggesting PRES. Ultimately, over a period of 4 days, his hypocalcemia could be corrected and the child was neurologically normal on the 5th day. CT scan of the...

Posterior reversible encephalopathy syndrome in setting of postobstructive diuresis and persistent hypocalcemia.

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Xanthogranulomatous pyelonephritis-mimicking renal mass in 5-month-old child.

Bilateral focal xanthogranulomatous pyelonephritis in a child presenting as complex cystic renal mass: a report on non-surgical treatment.

Posterior reversible encephalopathy syndrome in setting of postobstructive diuresis and persistent hypocalcemia.