Xanthogranulomatous Pyelonephritis in Korean Children

PURPOSE Xanthogranulomatous pyelonephritis (XGP) is rare among children. In most cases, XGP is diffusely or focally enlarged, mimicking the neoplastic process. The aim of this study was to examine clinical characteristics and outcomes of Korean children with XGP. MATERIALS AND METHODS Fourteen children (9 boys, 5 girls) with XGP were reviewed retrospectively. The cohort included 2 children managed at our institution and 12 children reported in the Korean literature. The patients' records were reviewed with respect to age at diagnosis, clinical presentation, management method, and other characteristic features. RESULTS The mean age was 79.4±66.5 months (range 1-168 months). Common clinical presentations included fever (85.7%), abdominal pain (57.1%), and palpable mass (28.6%). Laboratory abnormalities included leukocytosis (57.1%), anemia (57.1%), and pyuria (57.1%). The types of XGP that were diagnosed based on preoperative radiologic studies included the focal form in 9 children and the diffuse form in 5. Thirteen children underwent nephrectomy, and 1 child received conservative medical therapy. CONCLUSION The possibility of XGP should be considered if a child is diagnosed with a renal mass, especially if it is a small renal mass associated with fever, leukocytosis, or stone. Nephrectomy is the treatment of choice for the diffuse form, whereas partial nephrectomy or conservative medical therapy may be indicated to manage focal XGP.