Xanthogranulomatous pyelonephritis (XPN) is an unusual and severe form of chronic inflammatory lesion of the kidney. It is 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. Due to the fact that there are only few cases in the literature which describe the disease in children and since pathomechanism of XPN is poorly understood, this article aims to review and illustrate the clinical, radiological, surgical and pathological characteristics of XPN in children.
• 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.
Authors: Cinta Sangüesa Nebot, Sara Picó Aliaga, Agustín Serrano Durbá, María José Roca