Abstract
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.Teaching Points• 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.
Highlights
Xanthogranulomatous pyelonephritis (XPN) is an unusual, atypical 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, as well as inflammatory infiltration and intensive renal fibrosis.XPN is predominantly a disease of adults
There are no clear guidelines on the management of XPN
Conservative and surgical treatments should be considered for each individual case
Summary
Xanthogranulomatous pyelonephritis (XPN) is an unusual, atypical 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, as well as inflammatory infiltration and intensive renal fibrosis. XPN is predominantly a disease of adults In children it is diagnosed sporadically and is extremely rare in infants. The age of onset varies (21 days to 16 years), 60–75% of cases have been diagnosed before 5 years of age [1] It affects boys and girls and it is slightly more common in the left kidney. Two forms of XPN have been described: a diffuse form (which occurs in about 75% or 90% of cases) [5, 6] and a focal (or pseudo-tumoural) form, which is less frequent and more commonly described in children. The perirenal infiltration is possible in both types and can be mistaken for common renal neoplasm (Wilms’ tumour, clear cell carcinoma) or inflammatory process (renal or perirenal abscess, renal tuberculosis, focal or diffuse nephritis, sarcoidosis or Wegener granulomatosis disease).
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